Antiphospholipid syndrome leading to myocardial infarction in a Fontan patient 17 years after Norwood palliation.

Appropriate regimens for anticoagulation in the single ventricle population remain controversial and are usually based on individual practitioners' preferences. We present the case of a patient with a history of hypoplastic left heart syndrome and Norwood palliation who presented at 17 years of age with an acute myocardial infarction and a new diagnosis of antiphospholipid syndrome.

Sildenafil Increases Systemic Saturation and Reduces Pulmonary Artery Pressure in Patients with Failing Fontan Physiology.

OBJECTIVE: The purpose of this study was to investigate the effect of sildenafil in patients with failing Fontan physiology. DESIGN: A retrospective chart review was performed to compare history and available data in patients with Fontan circulations before and after starting sildenafil. The paired and unpaired Student's t-tests were used for statistical analyses. PATIENTS: Six patients at our institution with Fontan physiology, persistent symptoms of cyanosis or effusion, and poor hemodynamics as measured in the catheterization laboratory were placed on sildenafil. One patient was not included in the analysis because of insufficient length of treatment. All patients had symptoms of failing Fontan hemodynamics with either persistent cyanosis or effusions. In this group, the mean pulmonary artery pressure was greater than 15 mm Hg (17.4 ± 1.5 mm Hg) with mean estimated pulmonary vascular resistance of 3.5 ± 1.0 Wood units × m(2) prior to starting sildenafil. RESULTS: Sildenafil significantly increased the systemic arterial oxyhemoglobin saturation in this group (82.8 ± 7.3% pre-treatment vs. 91.0 ± 5.5% post-treatment, P = .017). In the four out of five patients who have had follow-up catheterizations, there was a significant decrease in pulmonary artery pressure (17.4 ± 1.5 mm Hg pre-treatment vs. 13.8 ± 2.1 mm Hg post-treatment, P = .018) and in estimated pulmonary vascular resistance pre- and post-sildenafil treatment (3.5 ± 1.0 Wood units × m(2) pre-treatment vs. 2.0 ± 0.4 Wood units × m(2) post-treatment, P = .031). CONCLUSIONS: Sildenafil may be a useful adjunct to therapy in patients with failing Fontan physiology likely through its function as a pulmonary vasodilator.

Interventional cardiac catheterization procedures in pediatric cardiac transplant patients: transplant surgery is not the end of the road.

OBJECTIVES: The objectives of this study are to report the spectrum of cardiac lesions in pediatric patients post-orthotopic heart transplantation (OHT), the characteristics of patients who develop these lesions, and the feasibility of transcatheter intervention in treating these lesions. BACKGROUND: Indications for OHT in the pediatric population range from cardiomyopathy to complex congenital heart defects with and without prior palliation. These patients may have residual vascular access and great vessel abnormalities. METHODS: Data was collected through retrospective review of all OHT patients at our institution from 1988 to 2005. RESULTS: During the study period, 276 heart transplants were performed. Forty-seven patients, age 1.6 [0.1-26] years with a weight of 9.5 [3.5-96.0] kilograms, underwent 69 procedures. Patients with original diagnoses of hypoplastic left heart syndrome and failed palliations required intervention most frequently. Sixteen patients, all with a history of left sided disease, developed aortic arch obstruction. Fifteen were successfully treated with balloon angioplasty while one has recurrent supravalve aortic obstruction. Fourteen patients had superior vena cava obstruction treated with balloon angioplasty and/or stent placement. Twelve patients required no further intervention and two required further stent placement. Ilio-femoral vein occlusion was treated with balloon angioplasty alone in 4 patients and stent placement in 10 patients to achieve vessel patency. Other procedures included treatment of branch pulmonary artery and pulmonary vein stenosis. CONCLUSION: Anastomotic aortic arch and venous obstructive lesions should be sought following pediatric OHT as they occur in almost 20% of patients and can be successfully addressed using interventional techniques.