The Heart Center - Arkansas Children's Hospital

www.archildrens.org


Vol 1 Issue 5	June 2006
Inside this issue
Changing the World of Hypoplastic Left Heart Syndrome
Acute Pericarditis, Pericardial Effusion and Cardiac Tamponade
The Thrombelastograph Analyzer and Coagulation Monitoring
Interns Join Heart Team for the Summer
ACH Heart Patients Have Opportunities to Play
Spotlight on Glena Martin, RT(R)
Spotlight on Lametria Williams, RN, BSN

Changing the World of Hypoplastic Left Heart Syndrome

Robert D.B. "Jake" Jaquiss, M.D., Chief, Pediatric and Congenital Cardiothoracic Surgery, Arkansas Children’s Hospital; Professor, Department of Surgery, University of Arkansas for Medical Sciences College of Medicine

"Never doubt that a few committed individuals can change the world. Indeed it is the only thing that ever has.” -- Margaret Mead

Hypoplastic left heart syndrome (HLHS) is a congenital heart malformation in which the left side of the heart and its attached structures are too small and underdeveloped to support normal circulation; in effect, the heart in HLHS has only one pumping chamber, as demonstrated in Figure 1. Of the congenital heart malformations, HLHS is among the most common, occurring in approximately 1,000 babies annually in the United States, but also among the most lethal without appropriate treatment. As many as 90 percent of babies with HLHS will die within 30 days of birth if appropriate therapy is not instituted. Unfortunately, until relatively recently, despite the ability to diagnose HLHS, there simply was no effective surgical or medical treatment available, and the diagnosis was essentially uniformly fatal.

This dismal outlook began to change in the early 1980s with the development of an operation by Dr. William Norwood that allowed some children with HLHS to survive. After the initial neonatal surgery, two subsequent operations (the superior cavopulmonary anastomosis and the Fontan operation) are necessary to achieve optimum surgical palliation for children with HLHS, typically completed at 3 to 6 months and 1.5 to 2 years, respectively. However, because of the technical difficulty of Norwood’s first-stage operation, the poor pre-operative condition of many babies, and the extraordinary fragility of HLHS in the immediate post-operative period, many children survived the initial operation only to succumb in the next few days. Because of these discouraging results in the early days of HLHS surgery, an alternative approach, that of neonatal heart transplantation, was developed by Dr. Leonard Bailey. This solution for HLHS babies was hampered by a severe neonatal organ donor shortage, which in one famous case led Bailey to transplant a baboon heart into a child with HLHS. (The baby died a few days later because the baboon donor was of the wrong blood type.)

In the ensuing two decades since the pioneering work of Norwood, Bailey and others, there has been an intense focus on improving the early and late outcomes for children undergoing surgery for HLHS. Norwood’s original operation has been modified and standardized, to the extent that heart transplantation is rarely necessary for newborns with HLHS. The pre-and post-operative care of HLHS infants has advanced remarkably, as the unique physiology of the HLHS patient before and after surgery has come to be better understood. Novel monitoring techniques have allowed the near elimination of the previously frequently observed sudden cardiovascular collapse episodes after surgery. The impact of these changes on survival after first-stage surgery is shown in figure 2. For babies who have been discharged from the hospital, the introduction into the home setting of the relatively simple techniques of having the parents weigh babies daily and record oxygen saturation daily has also greatly reduced the incidence of sudden death at home which formerly occurred in as many as 10 to 15 percent of survivors of stage I surgery. Improvements in the understanding of the physiology of children at the second and third stage operations, as well as improvements in the conduct of surgery and post-operative care at each of these subsequent stages, have additionally enhanced the overall outcomes which have come to be expected for HLHS.

The modern story of HLHS is an outstanding example of the truth of Margaret Mead’s observation; a group of people working with dogged persistence has in a few short years dramatically altered the world for babies born with HLHS. There is much work yet to be done in this area, but the progress already achieved now offers hope for HLHS babies and their families where once there was none.

Figure 1 Hypoplastic Left Heart Syndrome
The right atrium (RA) and right ventricle (RV) are normally formed. The left atrium (LA) and left ventricle (LV) are diminutive. Blood flow to the lungs is provided normally, pumped by the right ventricle. Blood flow to the body artery, the aorta (Ao) is provided through the patent ductus arteriosus (PDA), pumped by the right ventricle.

Figure 2 Improving Survival after Stage I Surgery for HLHS

Acute Pericarditis, Pericardial Effusion and Cardiax Tamponade

Parthak Prodhan, M.D., F.A.A.P.; Pediatric Critical Care Medicine and Cardiology, Arkansas Children’s Hospital; Assistant Professor, University of Arkansas for Medical Sciences College of Medicine

Background:
The pericardium, which encases the greater portion of the heart and proximal segments of the arteries and veins, consists of two layers: a serous visceral layer, which is intimately adherent to the heart and epicardial fat, and a fibrous parietal layer. The two layers are separated by a small quantity (15 to 50 mL) of serous fluid.

Pericarditis, pericardial effusion and cardiac tamponade are clinical problems involving the potential space surrounding the heart or pericardium. Acute pericarditis is the most common pathologic process wherein there is inflammation of the pericardium. This inflammation may lead to fluid accumulation around the heart which is called pericardial effusion. Cardiac tamponade is the hemodynamic result of increasing pericardial pressure due to fluid accumulation in the pericardial space. This complication may be fatal if it is not recognized and treated promptly.

Etiology:
Acute pericarditis may be caused by a variety of diseases affecting the pericardium. Pericardial effusion and cardiac tamponade may occur as part of the clinical course of acute pericarditis or as a separate process.
Idiopathic:
Infectious: Bacterial [Staphylococcus, gram-negative rods, Pneumococcus, Streptococcus, Haemophilus influenzae, Neisseria gonorrhoeae, Neisseria meningitides, Brucella melitensis, Francisella tularensis, Legionella pneumophilia, Borrelia burgdorferi, Myocoplasma] Viral (coxsackievirus, adenovirus, varicella, echovirus, influenza, cytomegalovirus, HIV, hepatitis B, mumps, infectious mononucleosis) Mycobacterial (Mycobacterium tuberculosis, Mycobacterium avium-intracellulare) Fungal (Histoplasma, Coccidioidomycosis, Blastomyces, Candida albicans, Nocardia, Actinomyces) Protozoal (Toxoplasma, Echinococcus, amebae) AIDS-associated
Neoplastic: Primary (fibrosarcoma) Secondary ( lymphoma, leukemia)
Immune/inflammatory: Connective tissue diseases (acute rheumatic fever, juvenile rheumatoid arthritis, systemic lupus erythematosus, dermatomyositis, Wegener's granulomatosis) Arteritis (polyarteritis nodosa, Takayasu's arteritis)
Iatrogenic: Postcardiotomy; Instrument/device trauma (implantable defibrillators, pacemakers, catheters Drugs (anticoagulants, phenytoin, hydralazine, daunorubicin, isoniazid, cyclosporine, dantrolene), post-cardiac resuscitation, post-radiation injury
Traumatic: Blunt trauma, penetrating trauma
Nephrogenic: chronic renal failure- uremia
Metabolic
Others: Aortic dissection, Acute myocardial infarction and post-MI (Dressler's syndrome) - rare in children
Congenital
Cardiac tamponade is more commonly seen as a result of penetrating cardiac injuries, in patients with malignant pericarditis. Iatrogenic causes may occur secondary to perforation due to central line placement, pacemaker insertion, cardiac catheterization, sternal bone marrow biopsies, and pericardiocentesis.

Acute Pericarditis
Symptoms: The primary symptom of acute pericarditis is the sudden or gradual onset of sharp or stabbing precordial or retrosternal that may radiate to the back, neck, left shoulder or arm. However, the location, intensity and nature can be very variable. Any movement, inspiration lying in supine position may aggravate the pain and can be relieved when the patient leans forward while sitting.

This type of typical pain is commonly seen in patients with acute infectious, hypersensitivity or autoimmunity causes for pericarditis. Pain is often absent in a slowly developing pericarditis (tuberculous, postirradiation, or uremia). Others may present with acute abdominal pain, low-grade intermittent fever, dyspnea, cough, and dysphagia, symptoms suggestive of the underlying systemic involvement from the disease or a prodromeal symptoms suggestive of a viral infection.

Physical Examination: Patients with pericarditis may be febrile and tachycardiac. Premature atrial and ventricular contractions may occasionally be present. The pericardial friction rub is the most characteristic finding of acute pericarditis. It has a high pitched scratchy character and may have up to three components per cardiac cycle corresponding to atrial contraction, ventricular systole and early diastole. However, sometimes only one or two components are heard. The systolic component is most consistently present. Sometimes it can be elicited only when firm pressure with the diaphragm of the stethoscope is applied to the chest wall at the left lower sternal border. It is heard most frequently during expiration with the patient in an upright and leaning forward position. Furthermore, because posture can affect the pericardial rub, auscultation with the patient in several positions, (supine, sitting, etc) is often helpful. Because the friction rub may be evanescent, varying widely in intensity even in the course of a single day, repeated auscultation during the course of the clinical course is important.

Pericardial Effusion
Symptoms: In acute pericarditis, development of effusion is usually associated with symptoms similar to those described above under pericarditis.

Physical Examination: Heart sounds tend to become faint with pericardial effusion; the friction rub of acute pericarditis may disappear. The apex impulse may vanish, but sometimes it remains palpable, albeit more medial to the left border of cardiac dullness. As fluid accumulates, the base of the left lung may be compressed by pericardial fluid, producing Ewart's sign, a patch of dullness beneath the angle of the left scapula. In addition, as fluid accumulates and impairs heart function, hepatomegaly and ascites may develop.

Cardiac tamponade:
The rapidity with which the clinical presentation unfolds depends on both the rapidity of accumulation and the volume of the effusion. Rapid clinical worsening may develop when fluid accumulates rapidly pericardial a small fluid volume (< 50 mL in small children). If there is slow fluid accumulation there can be as much as 2000 mL in older kids and adults, as the pericardium has had the opportunity to stretch and adapt to an increasing volume.

Symptoms: The clinical presentation of patients presenting with cardiac tamponade is influenced by the volume and rate of fluid accumulation in the pericardial space. Patients may present sub-acutely with symptoms of anxiety, dyspnea and fatigue. In more severe cases, consciousness may be impaired secondary to decreased cardiac output. A waxing and waning clinical picture may be present in intermittently decompressing tamponade. There may be symptoms related to the underlying medical illnesses.

Signs: The patient with tamponade is typically tachycardiac and tachypneic although bradycardia may ensue, especially in terminal stages. The systemic arterial pressure is typically low, although it may be surprisingly well-preserved on occasion; pulse pressure is usually diminished. Beck triad is classically present (jugular venous distention, hypotension, and muffled heart sounds) is classically seen with cardiac tamponade. Diminished heart sounds can be heard in one third of cases; a pericardial friction rub may be heard but is absent in the majority of patients. However, it is difficult to evaluate for jugular venous distension in children due to the presence of a short neck. In those in whom this pressure can be measured, it is usually markedly elevated, and the jugular venous pulse waveform reveals obliteration of the normal y descent. There may be signs of reduced cardiac output; shock may also be present.

Pulsus paradoxus is present in most cases. Pulsus paradoxus is measured objectively by careful auscultation with a blood pressure cuff. The first sphygmomanometer reading is recorded at the point when beats are audible during expiration and disappear with inspiration. The second reading is taken when each beat is audible during the respiratory cycle. A pulsus paradoxus greater than 10 mm Hg is considered abnormal. However, abnormal pulsus paradoxus may be absent in such clinical situations where tamponade coexists with severe atrial septal defect or aortic insufficiency or in obstructive airway disease. The absence of a pulsus paradoxus in these settings should not dissuade the physician from the correct diagnosis. This physical finding is not pathognomonic of pericardial disease as it may be observed in some cases presenting with hypovolemic shock, acute and chronic obstructive airways disease, and pulmonary embolus.

Diagnostic Studies
Evaluation of a patient with suspected pericarditis/ pericardial effusion should include tests to diagnose the clinical disease (echocardiography, an ECG, etc.) and to find the etiology (tuberculosis, uremia, etc).

Electrocardiography:
Serial ECG can be diagnostic in pericarditis and evolves in four stages. However, only 50 percent of patients with pericarditis experience all four stages. In stage I, there is widespread ST-segment elevation. The ST segment is concave upward (in distinction to the elevation in myocardial infarction). ST-segment elevation is typically present in all leads except aVR and V1, where ST-segment depression is present. The T waves are upright in the leads with ST elevation. These changes accompany the onset of chest pain. It can be differentiated from the normal variant of early repolarization by evaluating the ST:T ratio in V6. A ratio of less than four is more suggestive of pericarditis.

Another important ECG finding is PR-segment depression, which has been reported in up to 80 percent of viral pericarditis cases. This depression of the PR segment (below the TP segment) reflects atrial involvement. In stage II, typically occurring several days later, the ST segments return to baseline, and the initially upright T waves flatten. In stage III, the T waves invert, and the ST segments may become depressed—changes that may persist indefinitely. The stage IV the ECG normalizes. Electrical alternans is pathognomonic of cardiac tamponade and is characterized by alternating levels of ECG voltage of the P wave, QRS complex and T waves. This is a result of the heart swinging in a large effusion.

Chest radiography:
The chest radiograph is not very helpful in uncomplicated viral pericarditis. In cardiac tamponade (or large effusions), the chest X-ray may demonstrate an enlarged cardiac silhouette. However, the cardiac silhouette may be remarkably normal in size in cases where a modest sized effusion accumulates rapidly. Lucent pericardial fat lines may be seen deep within the cardiopericardial silhouette but does not necessarily indicate tamponade. Occasionally, the chest radiograph offers clues to important coexisting conditions, such as aortic dissection or malignancy.

Echocardiography:
Echocardiography is a sensitive, specific, simple, non-invasive test which may be performed at the bedside. In pericarditis, the pericardium may have a normal appearance, without evidence of fluid accumulation. It confirms the presence of pericardial fluid and is identified on echocardiography as a relatively echo-free space between the posterior pericardium and left ventricular epicardium in patients with small effusions and as a space between the anterior right ventricle and the parietal pericardium just beneath the anterior chest wall in those with larger effusions.

The most useful echocardiographic sign of increased intrapericardial pressure is diastolic collapse of the right atrium and right ventricle. Though these changes are neither completely sensitive nor specific, they first occur when the pericardial pressure transiently exceeds the intracardiac chamber pressure. They can therefore be useful in identifying patients whose pericardial pressure level should be of concern. Echocardiography is also an extremely useful tool to guide pericardiocentesis. A swinging heart may be present. This is characterized as counterclockwise rotational movement, which occurs in addition to the triangular movement of the heart, producing a dancelike motion. A dilated inferior vena cava (IVC) without inspiratory collapse (plethora) is highly suggestive of tamponade. However, sometimes the transthoracic echocardiography may be limited in its capacity to image the entire pericardium due to poor echo “windows.”

Cardiac Catheterization:
This test is rarely required to diagnose tamponade as echocardiography findings are mostly diagnostic. However, in the patient with tamponade, cardiac catheterization will reveal depressed cardiac output and elevated equal or near-equal filling pressures in all four chambers. Examination of the atrial pressure waveforms reveals the loss of the normal y descent.

Computed Tomography Scanning/ Magnetic Resonance Imaging:
The diagnosis of pericardial fluid or thickening may be confirmed by computed tomography (CT) or magnetic resonance imaging (MRI). These techniques may be superior to echocardiography in detecting loculated pericardial effusions and pericardial thickening. An advantage of CT scanning over other imaging modalities includes its capacity to detect pericardial calcifications which may be missed on MRI. The limitations of CT scanning include the need for contrast administration, patient exposure to ionizing radiation, and difficulty in differentiating fluid from thickened pericardium. MRI has the ability to provide anatomic details of the pericardium and heart without ionizing contrast or radiation. However, a high quality MRI may need more than 250 regular heartbeats to gate the image acquisition and therefore the examination may be limited in patients with arrhythmias.

Other Tests:
Laboratory evidence of inflammation, (CBC with mild leukocytosis, modestly elevated erythrocyte sedimentation rate, increased c-reactive proteins) may be present. Cardiac enzymes may be slightly elevated when the inflammatory process involves the sub-epicardial myocardium. Specific testing may be required to identify specific etiology (HIV testing, tuberculosis skin testing, thyroid function tests, antinuclear antibody, etc). Examination of pericardial fluid is imperative. Evaluation should include cell count and differential, and stains and culture for aerobic and anaerobic bacteria. Special tests for identifying cases with tuberculosis (acid-fast stain and culture), fungus (fungal stains and culture), and viral (cultures and viral nucleic acid detection assays) may be indicated in some cases. Cytology can detect neoplasms.

Treatment
Pericarditis:
The management of pericarditis is determined by the type of pericarditis or the pathogen suspected or identified. In the usual case of idiopathic acute pericarditis, treatment with any of the nonsteroidal antiinflammatory agents usually suppresses the clinical manifestations rapidly within 24 hours. When this measure fails to ameliorate symptoms, steroid therapy may be initiated, with a large initial dose of prednisone which is tapered over a week or two. If symptoms recur as the dose is lowered, the minimum dose that will suppress the illness should be maintained for one to two months and then tapered and discontinued. In the majority of patients, pericarditis resolves without sequelae with a single course of non-steroidal antiinflammatory. In a minority, however, a recurrence may develop over a period of weeks or months after the initial episode. The recurrences can be managed with repeated courses of non-steroidal or steroidal anti-inflammatory agents. In difficult cases of recurrent pericarditis, immunosuppressive therapy may be useful and in particular may reduce the necessity for long-term steroid therapy. Colchicine has demonstrated efficacy in the prophylaxis of recurrent pericarditis and should be considered in these patients. Pericardiectomy may be required in rare cases of frequent and severe recurrences despite aggressive medical therapy.

Antimicrobial therapy alone is seldom sufficient for treatment of purulent pericarditis. Initial treatment with vancomycin or clindamycin combined with cefotaxime or other third-generation cephalosporin will provide adequate coverage for most organisms when an bacterial infectious agent is suspected. In immunocompromised hosts or in pericarditis occurring post-cardiac or thoracic surgery, or if associated with an indwelling catheter or intracardiac device, resistant Gram-positive organisms, such as Gram-negative organisms (including Pseudomonas aeruginosa), methicillin-resistant Staphylococcus aureus which should be covered with vancomycin, an aminoglycoside and a third generation cephalosporin or extended spectrum penicillin with activity against Pseudomonas. Amphotericin B deoxycholate or lipid formulations is indicated when Candida and Aspergillus are suspected. Fluconazole, itraconazole or voriconazole may be considered, however clinical efficacy have not been studied. Candida pericarditis may require ultimately pericardectomy.

Bacterial purulent pericarditis is usually treated for three to four weeks duration. The duration of therapy may have to be extended if complications develop or comorbid conditions exist or an unusual pathogens or fungal pericarditis is identified. Nonsteroidal antiinflammatory agents, administered for two to twelve weeks, might be helpful in pericarditis associated with Histoplasma capsulatum. Purulent pericarditis associated with a severe inflammatory response may benefit from a one- to two-week course of steroids in addition to adequate antibiotic coverage. Viral pericarditis associated with enteroviruses and adenoviruses usually resolves with supportive therapy in three to four weeks. Immunocompromised patients with cytomegalovirus-associated pericarditis require treatment with antiviral therapy, like ganciclovir, for a two- to three-week induction period, followed by maintenance therapy for the duration of severe immunosuppression.

Pericardial effusion and cardiac tamponade:
If a small- to medium-sized pericardial effusion is present, the patient may have to be followed closely with serial echocardiography to evaluate its progression. If a large effusion is present, the stable patient may undergo an urgent pericardiocentesis or placement of a pericardial window surgically.

If there are signs suggestive of cardiac tamponade, intravenous fluids and pressors can be administered as temporizing measure until pericardiocentesis can be performed. These modalities usually will not significantly improve the clinical status, however, and should never be used in place of or allowed to interfere with prompt evacuation of the fluid.

Drainage of pericardial fluid is the cornerstone of therapy. Draining even modest amounts of fluid may result in striking improvement. Unstable patients require immediate treatment of the increase in pericardial pressure with pericardiocentesis.

Echocardiographically guided pericardiocentesis is now considered the procedure of choice for removal of pericardial fluid. Echocardiography, by confirming the presence of a sufficiently large volume of fluid in an anterior location, can decrease the risk of cardiac puncture. The presence of at least 1-cm of echo-free space anterior to the heart has been recommended as a guideline for the minimum volume of fluid that should be present before percutaneous pericardiocentesis is undertaken. In addition, the patient should be positioned in a semi-upright position to allow inferior pooling of the effusion. The procedure is ideally carried out in the intensive care unit, the cardiac catheterization laboratory or in the operating room. Utilizing a sub-xyphoid approach, the pericardial fluid can be drained.

Pericardial fluid can also be evacuated through a subxiphoid surgical pericardiotomy performed under local or general anesthesia. Further, open thoracotomy and pericardiotomy may be required if the patient has rapid deterioration or cardiac arrest. It also permits pericardial biopsy in select cases where the etiology is unclear. The pericardial fluid obtained can be sent for cultures and cytologic examination. In most cases the physician and surgeon may consider leaving a pericardial catheter in place for continued drainage. The catheter can be removed when the rate of drainage decreases. Rarely, definitive management of pericardial fluid accumulation may require surgical removal of the pericardium or the creation of an opening between the pericardium and left pleura.

In recent times a percutaneous balloon technique for creating a pleuropericardial opening has recently been described. Intra-pericardial fibrinolytic therapy with streptokinase has been used to liquefy thick pus in cases with purulent pericarditis. Intravenous immunoglobulin preparations are not beneficial for patients with acute infectious pericarditis.

Conclusion:
Acute pericarditis, pericardial effusion and cardiac temponade represent a spectra of continiium. This process is caused by a variety of diverse causes. Rapid accumulation of fluid in the pericardial fluid may require urgent management in a specialized center with coordination between specialists from different area.

References:
Goldstein JA: Cardiac tamponade, constrictive pericarditis, and restrictive
cardiomyopathy. Curr Probl Cardiol 2004 Sep; 29(9): 503-67

Vasquez A, Butman SM: Pathophysiologic mechanisms in pericardial disease. Curr Cardiol Rep 2002 Jan; 4(1): 26-32

Fowler NO: Cardiac tamponade: A clinical or an echocardiographic diagnosis? Circulation 1993;87:1738.

Dennis L. Kasper, Eugene Braunwald, Anthony S. Fauci, Stephen L. Hauser, Dan L. Longo, J. Larry Jameson, and Kurt J. Isselbacher, Eds. Part 8. Disorders of the Cardiovascular System Harrison's Principles of Internal Medicine, 16th Edition

Valentin Fuster, R. Wayne Alexander, and Robert A. O'Rourke, Eds. Pericardial diseases and infective endocarditis. Hurst's-The Heart 11th Edition.

Aikat S, Ghaffari S: A review of pericardial diseases: clinical, ECG and hemodynamic features and management. Cleve Clin J Med 2000 Dec; 67(12): 903-14

Palacios IF: Pericardial Effusion and Tamponade. Curr Treat Options Cardiovasc Med 1999 Jun; 1(1): 79-89

Spodick DH: Pericarditis, pericardial effusion, cardiac tamponade and constriction. Crit Care Clin 1989; 5: 455

Spodick DH: Differential diagnosis of acute pericarditis. Prog Cardiovasc Dis 1971; 14: 192

Demmler, Gail J. Infectious Pericarditis in Children. 2006; 25: 165-166

Levy P, et al. Etiologic diagnosis of 204 pericardial effusions. Medicine. 2003;82:385–391.

Cakir O, et al. Purulent pericarditis in childhood. J Pediatr Surg. 2002;37:1404–1408.

Roodpeyma S, et al. Acute pericarditis in childhood. Pediatr Cardiol. 2000;21:363–367.

Lubega S, et al. Heart disease among children with HIV/AIDS. Afr Health Sci. 2005;5:219–226.

Mok G, et al. Large pericardial effusions. Cardiol Young. 2003;13:131–136.

Ernst E. Cardiovascular adverse effects of herbal medicines. Can J Cardiol. 2003;19:818–827.

Wang Z, et al. CT and MR imaging of pericardial disease. Radiographics. 2003;23:S167–S180.

Kabukcu M, et al. Pericardial tamponade and large pericardial effusions. Tex Heart Inst J. 2004;31:398–403.

Estok L: Cardiac tamponade in a patient with AIDS: A review of pericardial disease in patients with HIV infection. Mount Sinai J Med 1998;64:312.

Fowler NO: Tuberculous pericarditis. JAMA 1991;266:99.

Heidenreich P: Pericardial effusion in AIDS: Incidence and survival. Circulation 1995;92:3229.

Oliva BP et al: Effect of definition on incidence of post infarction pericarditis. Is it time to redefine post infarction pericarditis? Circulation 1994;90:1537.

Rostand SG, Rutsky EA: Pericarditis in end-stage renal disease. Cardiol Clin 1990;8:701.

Spodick DH: Pericarditis in systemic diseases. Cardiol Clin 1990;8:709.

Antony SJ, Haas DW: Tuberculous pericarditis in an HIV-infected patient. Scand J Infect Dis 1995; 27(4): 411-3

Atwood JE, Osterberg L: Images in clinical medicine. Constrictive pericarditis. N Engl J Med 2000 Jul 13; 343(2): 106

Barbaro G, Fisher SD, Giancaspro G: HIV-associated cardiovascular complications: a new challenge for emergency physicians. Am J Emerg Med 2001 Nov; 19(7): 566-74

Bennett JA, Haramati LB: CT of bronchopericardial fistula: an unusual complication of multidrug- resistant tuberculosis in HIV infection. AJR Am J Roentgenol 2000 Sep; 175(3): 819-20

Breen JF: Imaging of the pericardium. J Thorac Imaging 2001 Jan; 16(1): 47-54

De Benedetti E, Didier D: Images in clinical medicine. Constrictive pericarditis. N Engl J Med 2000 Jul 13; 343(2): 107

Debehnke DJ: Cardiac-related acute infectious disease. In: Emergency Cardiac Care. 1st ed. 1994:463-88.

Donnelly LF, Kimball TR, Barr LL: Purulent pericarditis presenting as acute abdomen in children: abdominal imaging findings. Clin Radiol 1999 Oct; 54(10): 691-3

Estok L, Wallach F: Cardiac tamponade in a patient with AIDS: a review of pericardial disease in patients with HIV infection. Mt Sinai J Med 1998 Jan; 65(1): 33-9

Merce J, Sagrista Sauleda J, Permanyer Miralda G: Pericardial effusion in the elderly: A different disease?. Rev Esp Cardiol 2000 Nov; 53(11): 1432-6

Ristic AD, Seferovic PM, Ljubic A: Pericardial disease in pregnancy. Herz 2003 May; 28(3): 209-15

Gupta R, Munyak J, Haydock T: Hypothyroidism presenting as acute cardiac tamponade with viral pericarditis. Am J Emerg Med 1999 Mar; 17(2): 176-8

Indik JH, Alpert JS: Post-Myocardial Infarction Pericarditis. Curr Treat Options Cardiovasc Med 2000 Aug; 2(4): 351-356

Mastroianni A, Coronado O, Chiodo F: Tuberculous pericarditis and AIDS: case reports and review. Eur J Epidemiol 1997 Oct; 13(7): 755-9

Markovchick V, Duffens KR: Cardiovascular trauma. In: Rosen P, Barkin RM, et al, eds. Emergency Medicine Concepts and Clinical Practice. 3rd ed. 1992:439-59.

Keefe DL: Cardiovascular emergencies in the cancer patient. Semin Oncol 2000 Jun; 27(3): 244-55

Marinella MA: Electrocardiographic manifestations and differential diagnosis of acute pericarditis. Am Fam Physician 1998;57: 699.

Hauser AM: The emerging role of echocardiography in the emergency department. Ann Emerg Med 1989; 18: 1298-1303

Horowitz MS, Schultz CS, Stinson EB: Sensitivity and specificity of echocardiographic diagnosis of pericardial effusion. Circulation 1974 Aug; 50(2): 239-47

Imazio M, Demichelis B, Cecchi E: Cardiac troponin I in acute pericarditis. J Am Coll Cardiol 2003 Dec 17; 42(12): 2144-8

Karia DH, Xing YQ, Kuvin JT: Recent role of imaging in the diagnosis of pericardial disease. Curr Cardiol Rep 2002 Jan; 4(1): 33-40

Mayron R, Gaudio FE, Plummer D: Echocardiography performed by emergency physicians: impact on diagnosis and therapy. Ann Emerg Med 1988 Feb; 17(2): 150-4

Plummer D, Dick C, Ruiz E: Emergency department two-dimensional echocardiography in the diagnosis of nontraumatic cardiac rupture. Ann Emerg Med 1994 Jun; 23(6): 1333-42

Sagrista Sauleda J, Almenar Bonet L, Angel Ferrer J: The clinical practice guidelines of the Sociedad Espanola de Cardiologia on pericardial pathology. Rev Esp Cardiol 2000 Mar; 53(3): 394-412

Sagrista-Sauleda J, Merce J, Permanyer-Miralda G: Clinical clues to the causes of large pericardial effusions. Am J Med 2000 Aug 1; 109(2): 95-101

Sagrista-Sauleda J, Angel J, Permanyer-Miralda G: Long-term follow-up of idiopathic chronic pericardial effusion. N Engl J Med 1999 Dec 30; 341(27): 2054-9

Salem K, Mulji A, Lonn E: Echocardiographically guided pericardiocentesis - the gold standard for the management of pericardial effusion and cardiac tamponade. Can J Cardiol 1999 Nov; 15(11): 1251-5

Sechtem U, Tscholakoff D, Higgins CB: MRI of the abnormal pericardium. AJR Am J Roentgenol 1986 Aug; 147(2): 245-52

Shikama N, Terano T, Hirai A: A case of rheumatoid pericarditis with high concentrations of interleukin-6 in pericardial fluid. Heart 2000 Jun; 83(6): 711-2

Tsang TS, Freeman WK, Sinak LJ: Echocardiographically guided pericardiocentesis: evolution and state-of- the-art technique. Mayo Clin Proc 1998 Jul; 73(7): 647-52

Hakim JG, Ternouth I, Mushangi E: Double blind randomised placebo controlled trial of adjunctive prednisolone in the treatment of effusive tuberculous pericarditis in HIV seropositive patients. Heart 2000 Aug; 84(2): 183-8

Tsang TS et al: Echocardiographically guided pericardiocentesis. Evolution and state-of-the-art technique. Mayo Clin Proc 1998;73:647.

Adler Y et al: Colchicine treatment for recurrent pericarditis: A decade of experience. Circulation 1998;97:2183.

Becit N, et al. Clinical experience with subxiphoid pericardiostomy. J Int Med Res. 2003;31:312–317.

Ustunsoy H, et al. Intrapericardial fibrinolytic therapy. Eur J Cardiothorac Surg. 2002;22:373–376.

Peterlana D, et al. Efficacy of intravenous immunoglobulin. Clin Rheumatol. 2005;24:18–21. Epub July 14, 2004.

Adler Y, Finkelstein Y, Guindo J: Colchicine treatment for recurrent pericarditis. A decade of experience. Circulation 1998 Jun 2; 97(21): 2183-5

Adler Y, Guindo J, Finkelstein Y: Colchicine for large pericardial effusion. Clin Cardiol 1998 Feb; 21(2): 143-4

Marcolongo R, Russo R, Laveder F: Immunosuppressive therapy prevents recurrent pericarditis. J Am Coll Cardiol 1995 Nov 1; 26(5): 1276-9

Gibbs CR, Watson RD, Singh SP, Lip GY: Management of pericardial effusion by drainage: a survey of 10 years' experience in a city centre general hospital serving a multiracial population. Postgrad Med J 2000 Dec; 76(902):809-13

The Thrombelastograph Analyzer and Coagulation Monitoring

Juan L.Tucker MPS, C.C.P.; Assistant Chief Perfusionist, Pediatric Cardiovascular Surgery, Arkansas Children’s Hospital

The Thrombelastograph (TEG) is a non-invasive instrument designed to monitor and analyze the coagulation of blood samples. This instrument is used in evaluating and monitoring bleeding after cardiology procedures, trauma, organ transplantation,cardiovascular surgery and post operative hemorrhage.

TEG

The TEG analyzer is a clinical monitor designed to evaluate the interaction of platelets and plasma factors, plus the effects of other cellular elements (white blood cells, red blood cells, etc). The end result of the hemostasis process (blood clot formation) is the rate, strength, and stability of clots produced in the circulatory system. This will determine the patient’s ability not to bleed excessively or develop thrombosis (blood clots blocking arteries or veins) after trauma or surgery.

The computerized TEG creates a coagulation profile that measures the formation, dissolution and quality of clots. This profile requires two to three milliliters of blood sample for testing. Five coagulation parameters (R, K, A, MA, LY30) are evaluated in each profile.

The Reaction Time (R) represents the time from the start of a test until the first detection of a clot formation. The (R) times are prolonged by anticoagulation and factor deficiencies, but are shortened by hypercoagulable states.

The K Time (K) represents the beginning of clot formation until a fixed level of clot firmness is achieved. (K) Time measures the speed and strength of clot formation. (K) Time is reduced by increased fibrinogen levels and platelet function. (K) Time is prolonged by anticoagulants that affect both.
The Angle (A) represents the kinetics of clot formation and provides more comprehensive information than (K) Time. The Angle is larger with increased fibrinogen levels and good platelet function. The Angle size is reduced by anticoagulants that affect both.
The Maximum Amplitude (MA) measures the maximum strength of the developed clot. The clot strength is a result of fibrin and platelet function.
The (LY30) represent the percent of clot lysis at 30 minutes after maximum clot strength is reached. LY30 values are increased when fibrinolytic activity is high. Clotting

Blood sample preparations can be modified by adding reagents to improve the speed of analysis or to reverse heparinization. Activators such as celite, kaolin, tissue factor and thrombin can be added to native whole blood samples to speed the time of analysis.
    Celite (diatomaceous earth) reduce coagulation time by activating the intrinsic pathway. Kaolin (hydrated aluminum silicate) also activates the intrinsic pathways. Tissue factor is an enzyme that shortens coagulation time by activating the extrinsic pathway. Thrombin is an enzyme that shortens coagulation time by cleaving fibrinogen to form fibrin clots (common pathway).
    Heparinase and protamine can be added to neutralize the effects of heparin. Heparinase (Flavobacterium heparinum) is an enzyme that rapidly neutralizes the effects of heparin by cleaving heparin molecules into small inactive fragments not affecting other components involved in coagulation. Protamine (strongly cationic proteins) isolated from spermatozoa of spawn fish produces a stable complex which is void of anticoagulant activity.
    Anti-platelet drugs such as ReoPro (c7E3 Fab), inhibits clot retractions and abolishes platelet aggregation by binding fibrin receptors.
    Antifibrinolytic drugs such as amicar (aminocaproic acid), cyclokapron (tranexamic acid) and trasylol (aprotinin) can be used to limit excessive bleeding. Amicar is a mono-amino carboxylic acid that inhibits plasminogen activator substances. Tranexamic acid competitively inhibits the activation of plasminogen to plasmin. Aprotinin is a serine protease inhibitor that limits the function of plasmin and kallikrein.

TEG PlateletMapping Assay
The TEG analyzer can be used to assess platelet function in patients that receive platelet inhibiting drugs such as aspirin, plavix, reopro, aggrastat, persantine, etc. The PlateletMapping assay measures the presence of platelet inhibiting drugs using heparin, activatorF, ADP and arachidonic acid.
Principle

Thrombin is a direct activator of ( GP11b/111a ) platelet receptors. Adenosine-5-diphosphate (ADP) and thromboxane A2 (TxA2), mediate the activation of GP11b/111a receptors. These receptors are inhibited by drugs such as abciximab, tirofibin and eptifibatide. Aspirin can inhibit the enzyme cyclooxygenase which is needed in the production of TxA2. Clopidogreal inhibit ADP receptors. PlateletMapping assay measures the presence of platelet- inhibiting drugs using heparin, activatorF, ADP and AA (arachidonic acid).

Aspirin

None

% MA reduction = 100 – [(MAp – MA fibrin / MA thrombin – MA fibrin) X 100]

The test results are reported as the percent of maximum amplitude reduction (platelet inhibition) calculated by the TEG software. The presence of platelet inhibiting drugs is reflected in the reduction of maximum amplitude values. This test requires
two to three milliliters of blood sample to complete the assay. These results with clinical information are valuable in developing strategies for managing patient haemostasis.
The Haemoscope Decision Tree uses results from TEG analysis to identify the coagulopathy, primary and secondary fibrinolysis, platelet-induced or enzymatic hypercoagulability. Normal

This allows the clinician to use a systematic way of diagnosing a coagulopathy and, subsequently, developing a plan for treatment. The patient’s clinical status and bleeding state are important factors in the decision process.

Rudolph F, Ramsay KJ, Ramsay MAE, Hein HAT, Carnes K, Turner K. "Accelerators in [Thrombelastograph® Analysis]: Effects of Tissue Factor and Heparinase on the Thrombelastogram During Liver Transplantation." Anesth Analg. 1998;86;S228.
Gonano C, Kettner S, Bohm D, et al. "Detection of Heparin Effects with Heparinase Modified [Thrombelastograph® Analysis] (TEG[®]) in Liver Transplantation (OLT) without Veno-Venous Bypass." Anesthesiology. Sept. 1996;V85, No. 3A.
Von Kier S, Royston D. "Reduced Hemostatic Factor Transfusion using Heparinase-Modified [Thrombelastograph® Analysis] (TEG[®]) During Cardiopulmonary Bypass (CPB)." Anesthesiology. Sept. 1998, V89, No. 3A, A911.
Shore-Lesserson L, Manspeizer HE, Francis S, DePerio M. "[Thrombelastograph® Analysis] Decreases Transfusion Requirements after Cardiac Surgery." Anesthesiology. Sept. 1998, V89, No3A, A246.
Stammers AH, Bruda NL, Gonano C, Hartmann T. "Point-of-care coagulation monitoring: applications of the [Thrombelastograph®]." Anesthesia. 1998,53 (Suppl 2), pp 1-80.
Brown RS, Thwaites BK, Mongan PD. "Tranexamic Acid is Effective in Decreasing Postoperative Bleeding and Transfusions in Primary Coronary Artery Bypass Operations: A Double-Blind, Randomized, Placebo-Controlled Trial." Anesth Analg 1997;85:963-70.
Thwaites BK, Mongan PD, Brown RS. "[Thrombelastograph®] Evidence of Fibrinolysis Strongly Predicts Excess Bleeding After Cardiopulmonary Bypass." Anesth Analg. 1995;80;SCA1-SCA141.
Handa A, Cox DJ, Hamilton G, Pasi KJ, Perry DJ. "[Thrombelastograph® Analysis]: An effective screening test for prothrombotic states." Presented at XVIth Congress of the International Society on Thrombosis and Haemostasis. June 6-12, 1997.
Arcelus JI, Traverso CI, Caprini JA. "[Thrombelastograph® Analysis] for the Assessment of Hypercoagulability During General Surgery." Seminars in Thrombosis and Hemostasis. 1995:Suppl. 4, V21.
McCarthy RJ, Tuman KJ, Chen B, Ivankovich AD. "Platelet Integrin Inhibition with c7E3 Enhances the Correlation between Platelet Aggregometry and [Thrombelastograph®] (TEG[®]) MA Values." Anesth Analg. 1998;86;S219.
Miller BE, Tosone SR, Tam VKH, Kanter KR, Guzzetta NA, Bailey JM, Levy JH. "Hematologic and Economic Impact of Aprotinin in Reoperative Pediatric Cardiac Operations." Ann Thorac Surg 1998;6;535-41.
Chalkiadis GA, Gibbs NM. "The Effect of Aprotinin on [Thrombelastograph® Analysis] in vitro." Anaesth Instens Care. 1996;24:552-554.
Acimovic S, Stark J, Exner R, Kurz M, Bacher A, Spiss CK, Zimpfer M. "Effect of Platelet Count on [Thrombelastograph®] Maximum Amplitude: An in Vitro Trial." Anesthesiology. Sept. 1998, V89, No 3A, A964.
Spiess BD. "Coagulation Management Based Upon Whole Blood Coagulation Testing after CPB: [Thrombelastograph® Analysis] and Sonoclot." Presented at the Proactive Hemostasis Management: The Emerging Role of Platelets Symposium. Jan 23-24, 1997. Aspen, CO.

Pediatric Cardiac Critical Care Training at the Heart Center at Arkansas Children's Hospital: A Fellow's Perspective

Antonio Cabrera, M.D., Fellow, Cardiovascular Intensive Care Unit, Arkansas Children’s Hospital

The care of critically ill children with congenital and acquired heart disease requires knowledge and expertise in diagnosis, surgical techniques, specialized monitoring and advanced measures of life support. The discipline of Pediatric Cardiac Critical Care, a distinct field of relatively recent development, has developed combining the expertise of surgeons, anesthesiologists, intensivists, cardiologist and neonatologists.

Recent American College of Cardiology recommendations for training in Pediatric Cardiology specify that advanced training in Cardiac Intensive Care should be 9 months and should take place in an institution in which at least 250 pediatric cardiac procedures per year, utilizing cardiopulmonary bypass, are performed.

With more than 500 cardiac procedures per year, advanced training in Cardiac Intensive Care at the Heart Center is designed to provide that advanced expertise to board-eligible pediatric cardiologists or pediatric intensivists. The 12-month curriculum is divided so that about 70 percent of the year is spent in the Cardiovascular Intensive Care Unit caring for patients preoperatively and postoperatively. A separate month of Cardiac Anesthesiology is included to improve the understanding of cardiopulmonary bypass, innotropes, vasodilators and anesthetic agents. Also during this month, the fellow, as part of the cardiac anesthesia team, assists on the preoperative interview, arterial and central venous line placement and airway management. Additional expertise is gained on ultrasound-assisted line placement and transesophageal echocardiography.

During the fellowship, the trainee participates first-hand in the care of children who require Extracorporeal Membrane Oxygenation (ECMO), Ventricular assist devices (especially Berlin heart) and pre/postoperative heart transplantation. Heart transplantations at the heart center totaled 17 last year. Of key importance is rotation through the 26-bed Pediatric Intensive Care Unit, much awarded for excellence in care, infection control and superb design.

Although the fellowship is intended to be mostly clinical, the divisions of cardiology, critical care and pediatric cardiac anesthesia are heavily involved in research. There are currently more than 50 approved research protocols in the division of pediatric cardiology and multiple opportunities for involvement in research projects.

In addition, the fellow is on call with in-house attending physicians, which further enhances the learning experience and provides continuous feedback in a timely fashion.

The Cardiovascular Intensive Care at the Heart Center at ACH has assembled not only a superb group of expert clinicians, surgeons, nurses and nurse practitioners, but the most compassionate team that I have ever trained with. I suggest you visit our website http://www.uams.edu/pediatrics/fellowships/fellowships.asp

Starting July 1, 2006, Dr. Cabrera will be the medical director of the Cardiac Intensive Care at Le Bonheur Children’s Medical Center/University of Tennessee in Memphis.

Interns Join Heart Team for the Summer

This summer, three interns who are second-year medical students at the University of Interns Arkansas for Medical Sciences have joined the Heart Center team. Eric Wright of Quitman, Amy Taylor of North Little Rock and Amy Butenschoen of Little Rock are learning first-hand about providing care for pediatric heart patients.

Wright will be working with Dr. “Jake” Jaquiss and Dr. Erik Edens to research the outcomes of cardiac transplants in patients with pre-formed anti-HLA antibodies, which can be assessed by determining the panel reactive antibody level.

Taylor will be working with Dr. Jaquiss, as well as Dr. Jeffery Kaiser to research medical records, comparing the medical and surgical treatment for a Patent Ductus Arterisus (PDA). The goal is to be able to determine the best protocol to follow regarding the initial treatment of PDA.

Butenschoen will work with Dr. Jaquiss and Dr. William Fiser on a project involving long-term follow-up by telephone interview of children receiving aortic valve replacement (AVR) with a mechanical valve prosthesis.

ACH Patients Have Opportunities to Play

Morrow Children who visit ACH with heart problems are invited to attend Jake Heart Camp during the week of July 16-21. Those ages 6 to 18 years old are eligible for the event, held at Camp Aldersgate in Little Rock. They will be camping with children who visit the nephrology and arthritis clinics and will be able to fish, canoe and ride paddle boats. They can also participate in an ice cream social, a carnival night, adventure challenges, a dance, arts and crafts and even play music. The camp is partially funded by various organizations. For more information on Heart Camp, visit www.campaldersgate.net .

Children who visit the Heart Center also had a chance to visit with each other at the Little Rock Zoo recently. The Heart Center hosted its inaugural Heart Picnic, which will become an annual event. Patients were able to see their doctors and nurses, as well as the animals at the Zoo. The Heart Center even paid for each child to have a ride on the ACH Ticker Train. Check back next year for details about the 2007 Heart Picnic!

Spotlight on Glena Martin, RT (R)
Lead Tech/Lab Coordinator for the Cardiac Catherization

What is your role at ACH and how long have you worked here?
I am a registered radiology technologist. I am presently the Lead Tech/ Lab Coordinator of the cardiac catheterization lab at ACH.

Why is your job rewarding?
I have come into contact with so many children and their families. The opportunity to be involved in their care, both short and long term is truly a privilege.

How did you become interested in pediatric cardiology or cardiovascular surgery?
In the past I had the opportunity to work with children while working in Alaska as a cath lab tech, looking for a chance to return to Little Rock to work. I applied to ACH cath lab for a position. At that time, a lot of the Interventional procedures were coming into vogue, and I viewed this as a truly dynamic and educational time.

What do you want people to know about the Heart Center at Arkansas Children's Hospital?
The continuity of care and the amazing courage of the children and parents. We have over the years made an immeasurable impact on so many families.

What do you enjoy most about working with children?
I think, ultimately, their sheer tenacity. The fact that under very difficult circumstances they always seem to bounce back so very quickly. It’s the smiles; the gratitude and the awe with which they perceive the world.

What has been your most memorable moment working in the Heart Center at Arkansas Children's Hospital?
It’s all memorable – truly. It’s the rapid progress in technology mixed with, and perhaps more importantly, the human touch we are able to provide to both the children and their families in our cath lab.

What is your greatest professional achievement?
Several others and I were able to open a brand-new, state-of-the-art, digital cath lab (1997). Although this was a stressful time, it was also a learning experience. This and the position I presently hold makes me so very proud.

Spotlight on Lametria Williams, RN, BSN
CVICU RN

What is your role at ACH, and how long have you worked here?
I am a Registered Nurse in the Heart Center, and I have worked here for four years.

Why is your job rewarding?
My job is rewarding because children and their families are allowed more time together by what God enables us to do each and every day.

How did you become interested in pediatric cardiology or cardiovascular surgery?
My grandmother introduced me to Dr. Fontenot and Tammy Webb. Tammy interviewed me and offered me a job. Cardiovascular was my least favorite subject in school. I took the job as a sign that this was where I needed to be.

What do you want people to know about the Heart Center at Arkansas Children's Hospital?
Despite all the changes our unit has encountered we are still a great team and dedicate each day to changing someone’s life. We all have one thing in common,… our patients.

What do you enjoy most about working with children?
The thing I enjoy most is seeing them go home with their families. I’ve always wanted to be a pediatric nurse because kids make your job fun … and you never know what they will say next.

What has been your most memorable moment working in the Heart Center at Arkansas Children's Hospital?
My most memorable moment was seeing Dr. Michi Imamura asleep in the recliner at his patient’s bedside. It was his first surgery here at ACH. The patient was very critical and Dr. Michi didn’t want to leave the baby’s side. Of course, he didn’t sleep and probably didn’t eat all night. I felt relieved to know that Dr. Michi was there and ready to take care of any possible situation. He’s the man!

What is your greatest professional achievement?
My greatest professional achievement has been this: the ability to keep my faith in God, a sound mind and to know that every day I do my best. I cannot save the world, but as a nurse, I can do my part to make someone else’s life worth living.