Faculty Bibliography

BACKGROUND: Heart-lung transplantation has been recommended for patients with end-stage congenital heart disease (CHD) and single-lung physiology due to either discontinuous pulmonary arteries (PAs) and unilateral PA hypertension (HTN) or absence of 1 PA. METHODS: Eleven patients with CHD and single-lung physiology underwent heart transplantation (HT). Diagnoses included: tetralogy of Fallot, absent left PA (n = 4); single-ventricle s/p classic Glenn (n = 7), with absent left PA (n = 1); and severe left PA HTN (n = 6). RESULTS: Mean time from last surgery was 13 +/- 8 years; mean number of operations (op) was 3.2 +/- 1.7. Mean age was 21 +/- 11 years (range 9.5 to 43). Complications and procedures before HT included hemoptysis (n = 2), plastic bronchitis (n = 1) and interventional catheterization (n = 6). Mean cardiopulmonary bypass and ischemic time was 275 +/- 72 and 268 +/- 75 minutes, respectively. Mean time to extubation was 4.6 +/- 3.2 days, and mean length of stay was 19 +/- 7 days. Post-operative morbidity included bleeding (n = 4), vocal cord paralysis (n = 1) and coil embolization of aortopulmonary collaterals (n = 3). Early post-operative survival was 82%. Cause of death was aortic rupture (n = 1) and bleeding (n = 1). Eight patients are alive 4 years (range 0.9 to 7.6) after HT. PA continuity was established in 6 patients; post-HT lung perfusion scan showed no increase in perfusion to the left PA. One patient died from rejection 3 years post-HT. CONCLUSIONS: HT can be performed successfully in patients with single-lung physiology. HT is the procedure of choice in patients with end-stage CHD and a physiologic single lung

OBJECTIVES: We evaluated the early and medium-term single-center results for primary repair of Ebstein anomaly in both adults and children. METHODS: The records were reviewed of patients undergoing repair of Ebstein anomaly at the Children's Hospital of New York from September 1990 to September 2002. Functional, demographic, and echocardiographic parameters were studied both preoperatively and postoperatively, along with functional status and adverse events. The repair technique involved vertical plication of the atrialized ventricle and valve leaflet reimplantation after clockwise rotation. RESULTS: A total of 25 patients (19 children and 6 adults) underwent repair. The average age was 14.2 +/- 15.9 years, and the average follow-up was 4.1 +/- 3.4 years. Three patients required reoperation for right ventricular overload (1 child) and progressive, severe tricuspid regurgitation (2 adults); both adults received tricuspid valve replacements, one at 4 years and the other at 8 years post-repair. Three patients had radiofrequency ablation procedures performed intraoperatively. Ten patients (40%) had moderate-to-severe tricuspid regurgitation perioperatively. However, 18 children (95%) and 5 adults (83%) demonstrated significant improvement in exercise capacity late postoperatively. Two children died suddenly 11 months and 4 years after repair. DISCUSSION: Ebstein repair has good functional outcomes in children despite residual tricuspid regurgitation, likely because of reduction in right ventricular volume loading and relative annular and ventricular plasticity. Adult patients did not demonstrate the same durability of valve repair and frequently required tricuspid valve replacement. Intraoperative radiofrequency ablation represents an important adjunctive treatment for intractable arrhythmias, which may now represent relative indications for operative intervention

We describe an extremely rare case of primary cardiac precursor B lymphoblastic lymphoma (B-LBL) in a 10-year-old boy who presented with nonspecific complaints of fatigue and vomiting for 2 weeks and an episode of syncope. Chest X-ray showed cardiomegaly and echocardiography revealed a large right atrial mass, which was successfully resected. Pathology showed precursor B-LBL arising in the heart and there was no evidence of disease at any extracardiac site. A brief review of the literature is also presented

BACKGROUND: Nitric oxide (NO) binds to mitochondrial cytochrome oxidase to decrease myocardial oxygen consumption (MVO(2)). This regulation is disrupted in heart failure (HF) due to reduced NO. The present objective was to evaluate NO-mediated regulation of mitochondrial respiration in the myocardium of patients with congenital heart disease (CHD) and cardiomyopathy (CMP). METHODS: MVO(2) was measured in vitro in explanted human myocardium obtained at transplantation. Seven patients had CHD (5 cyanotic, 2 acyanotic), and 11 had non-ischemic CMP. The effects of the following on MVO(2) were measured: kinin-dependent endothelial NO synthase (eNOS) agonists, bradykinin, ramiprilat and amlodipine; NO donors, nitroglycerin and S-nitroso-N-acetylpenicillamine (SNAP) (10(-7) to 10(-4) mol/liter); and NOS inhibitor, N(omega)-nitro-L-arginine methylester (L-NAME). RESULTS: eNOS agonists caused a smaller decrease in MVO(2) in CHD compared with CMP patients. Changes in MVO(2) at the highest dose in CHD vs CMP were, respectively: bradykinin, -22 +/- 7% vs: -30 +/- 5% (p < 0.05); ramiprilat, -17 +/- 8% vs -26 +/- 2%, (p < 0.001); and amlodipine, -5 +/- 7% vs -29 +/- 6% (p < 0.001). L-NAME attenuated the effect of bradykinin, ramiprilat and amlodipine in both groups, confirming that the drug effect was secondary to eNOS activation. Nitroglycerin and SNAP also caused smaller decreases in MVO(2) in CHD vs CMP (NTG -16 +/- 6% vs -37 +/- 4%, SNAP -37 +/- 4% vs -49 +/- 3%, [p < 0.01]), suggesting altered mitochondrial function in CHD. CONCLUSIONS: Abnormal regulation of MVO(2) in end-stage CMP may be secondary to reduced endogenous NO availability and can be reversed by the use of NO agonists. In end-stage CHD, this abnormality may be related in part to abnormal mitochondrial function

BACKGROUND: This study investigated changes in left ventricular (LV) geometry and systolic function after corrective surgery for atrial (ASD) and ventricular septal defects (VSD). METHODS: Transesophageal LV short-axis echocardiograms were recorded before and after operative repair of ASD (n = 11) and VSD (n = 7). Preload was measured using LV end-diastolic area indexed for body surface area. Measurements of septal-freewall (D1) and anterior-posterior (D2) endocardial diameters were used to assess LV symmetry from D1/D2. Systolic indices included stroke area, area ejection fraction, and fractional shortening. RESULTS: Preload, stroke area, area ejection fraction, and fractional shortening of D1 increased after ASD repair but decreased after VSD repair (p < 0.05). End-diastolic symmetry increased after ASD closure and decreased after VSD closure (p < 0.05). Increases in stroke area and ejection fraction after ASD correction primarily reflected increased shortening of D1. A positive correlation was found overall between percent change in end-diastolic area (EDA) and percent change in area ejection fraction (r(2) = 0.80, p < 0.0001, n = 18). CONCLUSIONS: Preload was the primary determinant of changes in LV function in this series of ASD and VSD repairs. Intraoperative changes in position of the interventricular septum affected systolic and diastolic LV symmetry and septal free wall shortening. Additional studies are needed to define changes in afterload and contractility as well as diastolic compliance and systolic mechanics

BACKGROUND: We sought to introduce sodium-hydrogen exchange inhibition as prophylaxis against the development of ventricular dysfunction in the setting of implantable cardioverter defibrillator insertion in high-risk patients. Cariporide, shown to be safe in humans, was used to reproduce previous results in our laboratory that demonstrated that sodium-hydrogen exchange inhibition preserves left ventricular (LV) function after ventricular fibrillation (VF) and reperfusion. METHODS: Twelve pigs (weight, 35 to 55 kg) were divided into two groups of six. Baseline ventricular function studies were based on echocardiography, conductance, aortic flow, and LV pressure. Animals were given vehicle (control) or cariporide (3 mg/kg intravenously). Ten minutes later, hearts underwent 80 seconds of VF. After reperfusion for 40 minutes, function studies were repeated. RESULTS: Postmortem examination included measuring passive pressure-volume curves and myocardial water content. Systolic indices, including preload recruitable stroke work and ejection fraction, were significantly depressed from baseline after VF and reperfusion for control animals (preload recruitable stroke work, 30.13 +/- 0.59 [standard error of the mean] versus 43.85 +/- 2.60 mm Hg; ejection fraction, 25.7% +/- 2.4% versus 33.5% +/- 3.0%) but not for those in the cariporide group (preload recruitable stroke work, 38.36 +/- 1.87 versus 40.86 +/- 1.45 mm Hg; ejection fraction, 33.9% +/- 3.5% versus 32.8% +/- 3.9%). In vivo diastolic indices demonstrated trends toward diminished ventricular compliance in control animals but not in the cariporide group after VF and reperfusion. Control animals had significantly increased postmortem LV stiffness, myocardial water content, and normalized LV mass. CONCLUSIONS: Cariporide preserves LV function after 80 seconds of VF and 40 minutes of reperfusion. Cariporide may prove useful in patients with severe LV dysfunction undergoing VF for implantable cardioverter defibrillator testing