David Majdalany

Three-Dimensional Echocardiographic Assessment of Right Ventricular Volume and Function in Adult Patients With Congenital Heart Disease: Comparison With Magnetic Resonance Imaging

BACKGROUND The aim of this study was to evaluate the accuracy of three-dimensional (3D) ultrasound compared with the standard magnetic resonance imaging method in determining right ventricular (RV) volumes and function in adult patients with congenital heart disease and chronic, severe pulmonary regurgitation (PR). METHODS Twenty-five patients with severe PR secondary to either pulmonary valvotomy or tetralogy of Fallot repair were evaluated using 3D ultrasound and MRI. RESULTS The mean RV ejection fractions were 42 +/- 8% on 3D ultrasound and 44 +/- 7% on MRI (r = 0.89, P < .0001). The mean end-diastolic volumes were 249 +/- 66 and 274 +/- 82 mL and the mean end-systolic volumes 147 +/- 50 and 159 +/- 60 mL on 3D ultrasound and MRI, respectively. Similarly, there were strong correlations of both end-diastolic volume and end-systolic volume on 3D ultrasound and MRI (r = 0.88 and r = 0.89, respectively). CONCLUSIONS Three-dimensional ultrasound was comparable with MRI in determining RV size and function in most patients with complex congenital heart disease. It will be important to study 3D US in a larger population of patients with TOF, which will be possible only through multi-center collaboration.

Isolated partial anomalous pulmonary venous connections in adults: twenty-year experience.

OBJECTIVE We sought to review our experience with isolated partial anomalous pulmonary veins as their management is controversial. DESIGN We reviewed all patients with isolated partial anomalous pulmonary veins >18 years attending the Congenital Clinic from 1988-2008. RESULTS Forty-three patients aged 20-73 years were included. Fifteen patients had no surgery, 11 of whom had a single anomalous pulmonary vein. Surgical repair was performed in 28 patients: 27 had right ventricular volume overload, 21 had more than one anomalous pulmonary vein, and 1 had stenosed pulmonary veins. Right ventricular systolic pressure was elevated (>35 mm Hg) in 12 patients. Eleven patients had anomalous left pulmonary veins: 10 had the pulmonary vein anastomosed to the left atrial appendage and one patient had anastomosis to the left lower pulmonary vein. One patient had bilateral anomalous pulmonary veins anastomosed directly to the left atrium. Sixteen patients had anomalous right pulmonary veins: fifteen had a baffle through a surgically-created atrial septal defect, while one had the anomalous pulmonary vein anastomosed to the left atrium. There was no early mortality. During mean echo follow-up of 2.7 years, 19 patients (68%) had reduced right ventricular size and five had improved right ventricular function. Seven patients with increased pulmonary pressure had a decrease post-operatively. CONCLUSION Partial anomalous pulmonary veins can cause symptoms, right ventricular volume overload, and pulmonary hypertension, particularly when more than one pulmonary vein is anomalous. Surgical repair can be accomplished with low morbidity and mortality, with improvement in the right ventricular size and pulmonary pressures in most.

Adults with Down syndrome: safety and long-term outcome of cardiac operation.

OBJECTIVE As limited data exist, we sought to review the safety and outcome of cardiac surgery in the adult Down syndrome population. DESIGN We reviewed the data of all patients >or=18 years old with Down syndrome who underwent cardiac surgery (1969-2008) at our hospital. RESULTS Fifty patients underwent 57 surgeries (mean age 33 years). Fifteen patients had prior cardiac operations in childhood: 7 complete and 1 partial atrioventricular canal, 2 secundum atrial septal defect, 2 Tetralogy of Fallot, 1 patent ductus arteriosus, 1 combined atrioventricular canal and Tetralogy of Fallot, and 1 ventricular septal defect. Operations in adult Down syndrome patients included repair of partial atrioventricular canal in 17, aortic valve replacement in 7, mitral valve replacement/repair in 7, ventricular septal defect in 6, atrial septal defect in 3, Tetralogy of Fallot in 3, pulmonary valve replacement in 3, and other in 11. There was 1 in-hospital death (1.8%) from multiorgan failure. The mean hospital stay was 10.6 days. Average ventilatory support was 2.4 days (range 0-32). Atrial arrhythmias occurred in 14 patients (25%). Six patients had early postoperative pulmonary infections. Mean follow-up was 6 years, maximum 29 years. There were eight late deaths at an average age of 52 years (range 30-58) occurring 15 years postoperatively (range 32 days-29 years); two in the setting of dementia (mean age 56 years). CONCLUSION At an experienced center, adult patients with Down syndrome can undergo cardiac surgery with a low risk of mortality and acceptable morbidity. Atrial arrhythmias and pulmonary infections are common postoperatively.

Inferior wall myocardial infarction caused by anomalous right coronary artery

A 50-year-old man without previous coronary disease presented with an inferior myocardial infarction following exercise. He was initially treated with thrombolytic therapy and nitroglycerin. Subsequent coronary angio-graphy and cardiac computed tomography demonstrated an anomalous right coronary artery originating from the left coronary sinus and passing between the aorta and main pulmonary artery. The coronary arteries were otherwise patent. The patient later underwent transaortic unroofing of the anomalous right coronary artery and was discharged in good condition.