Achiau Ludomirsky

The Influence of a Restrictive Atrial Septal Defect on Pulmonary Vascular Morphology in Patients with Hypoplastic Left Heart Syndrome

Hypoplastic left heart syndrome (HLHS) with a restrictive atrial septal defect (ASD) is a form of congenital heart disease with considerable morbidity and mortality. This morphologic analysis assesses the pulmonary vasculature in this patient population. Pulmonary arteries, the persistence of high-resistance fetal arterioles, pulmonary veins, and lymphatics from multiple lung sections from each of five patients with HLHS and a restrictive ASD were compared to those of five patients with HLHS and nonrestrictive ASD. Lung sections from each patient were qualitatively graded in severity of pathology from 0 to 3 for each of the structures described previously, with the pathologist blinded to the status of the ASD. Patients with a restrictive ASD exhibited more significant pulmonary venous thickening and lymphatic dilatation (p = 0.02), with a tendency toward persistence of high-resistance fetal vessels (p = 0.2), compared to patients with a nonrestrictive ASD. These findings imply that patients with HLHS and a restrictive ASD possess pulmonary vascular abnormalities that place them at higher risk for the current surgical interventions available compared to patients with a nonrestrictive ASD.

Comparison of right ventricle to pulmonary artery conduit and modified Blalock-Taussig shunt hemodynamics after the Norwood operation

The Norwood procedure remains one of the highest-risk operations in congenital heart surgery. A significant contributor to this risk is thought to be the diastolic run-off into the modified Blalock-Taussig shunt (MBTS). In an effort to eliminate this risk, several groups have begun to utilize a right ventricle to pulmonary artery conduit (RVPAC), which decreases this diastolic steal of coronary blood flow. Whereas initial results with the RVPAC are encouraging, the postulated hemodynamic advantages are unproven. This case illustrates the positive hemodynamic changes by echocardiography after the replacement of a MBTS with a RVPAC in a patient after a Norwood procedure.

MR visualization of aortic valve vegetations

PURPOSEnThe purpose of this report is to describe the MR findings of aortic vegetations in two patients.nnnMETHODnMR imaging using SE and fast GE (Fastcard) sequences was performed in the axial and coronal planes.nnnRESULTSnThe vegetations were not visible with SE MRI, but were clearly visible with fast GE imaging (Fastcard). The vegetations appeared as areas of low signal at valve leaflets in contrast to the bright flowing blood.nnnCONCLUSIONnMR evaluation in patients with infective endocarditis is useful in clarifying echocardiographic findings and establishing the diagnosis in previously undiagnosed patients.

Evaluation of a noninvasive index of global ventricular function to predict rejection after pediatric cardiac transplantation

A Doppler myocardial performance index, defined as the sum of the isovolumetric contraction and relaxation time divided by the ejection time (ICT + IRT/ET), reflects global cardiac function, and when applied to the left ventricle, may serve as a predictor of moderate rejection in pediatric cardiac transplant patients.

Left Ventricular Outflow Tract Pseudoaneurysms in Congenital Heart Disease

complications of surgical and Amplatzer device closure of atrial septal defects. J Thorac Cardiovasc Surg 1999;118:674–680. 3. Sievert H. Interventional versus surgical closure of atrial and ventricular septal defects: advantages and limitations of the catheter-based approach. J Intervent Cardiol 2000;87:116–118. 4. Cao QL, Du ZD, Joseph A, Koenig P, Heitschmidt M, Rhodes J, Hijazi ZM. Immediate and six-month results of the profile of the Amplatzer septal occluder as assessed by transesophageal echocariography. Am J Cardiol 2001;88:754–759. 5. Masura J, Gavora P, Formanek A, Hijazi ZM. Transcatheter closure of secundum atrial septal defects using the new self-centering Amplatzer septal occluder: initial human experience. Cathet Cardiovasc Diagn 1997;42:388–393. 6. Rhodes J, Hijazi ZM, Marx GR, Fulton DR. Aerobic exercise function of patients with persistent coronary artery aneurysms secondary to Kawasaki disease. Pediatr Cardiol 1996;17:226–230. 7. Beaver WL, Wasserman K, Whipp BJ. A new method for detecting anaerobic threshold by gas exchange. J Appl Physiol 1986;60:2020–2027. 8. Jones NL. Clinical Exercise Testing. Philadelphia, PA: WB Saunders, 1988: 174. 9. Clark AL, Poole-Wilson PA, Coats AJS. Effects of motivation on indices of exercise capacity in chronic heart failure. Br Heart J 1994;71:162–165. 10. Wasserman K, Hansen JE, Sue DY, Whipp BJ, Casaburin R. Prinicples of Exercise Testing and Interpretation. 2nd Edition. Philadelphia, PA: Lea & Febiger, 1994:66–133. 11. Cumming GR. Maximal exercise capacity of children with heart defects. Am J Cardiol 1978;42:613–620. 12. Goldberg SJ, Mendes F, Hurwitz R. Maximal exercise capability of children as a function of specific cardiac defects. Am J Cardiol 1969;23:349–353. 13. Schofield PM, Barber PV, Kingston T. Preoperative and postoperative pulmonary function tests in patients with atrial septal defect. Br Heart J 1985;54: 577–582. 14. Porter CJ, Feldt RH, Edwards WD, Seward JB, Schaff HV. Atrial septal defects. In: Emmanouilides GC, Riemenschneider TA, Allen HD, Gutgessell HP, eds. Heart Disease in Infants, Children and Adolescents. 5th Edition. Baltimore, MD: Williams and Wilkins, 1995:687–703. 15. Wasserman K. New concepts in assessing cardiovascular function. Circulation 1988;78:1060–1071.

Amplatzer Closure of Atrial Septal Defect and da Vinci Robot-Assisted Repair of Vascular Ring

Technology for minimally invasive approaches to congenital heart disease is a rapidly evolving field. This case report reviews a novel approach to combining two of the newer technologies available to treat a pediatric patient with an atrial septal defect (ASD) and a vascular ring. This report is the first to describe the use of the da Vinci surgical system to assist in a thoracoscopic procedure for a pediatric patient. The da Vinci assisted division of the vascular ring, joined with an Amplatzer closure of the ASD, demonstrates how maximum benefit can be obtained for patients by combining emerging technologies.

Acute Myocardial Ischemia in a Healthy Male Child: An Atypical Presentation of Acute Epstein-Barr Virus Infection:

to pediatric emergency departments.1 Despite the fact that chest pain often evokes anxiety in patients and parents, it rarely represents significant pathosis. Furthermore, amidst the vast differential diagnosis of childhood chest pain, cardiac causes are rare and represent only 1% to 4% of cases. Nonetheless, it is important to consider the possible cardiac causes of chest pain when evaluating a child with this complaint because of their potential severity. We present a 14-year-old male who presented with chest pain who was found to have cardiac enzyme elevations and electrocardiographic evidence of ischemia associated with acute Epstein-Barr virus infection. Patient Report