Isolated left ventricular (LV) apical hypoplasia is a rare congenital cardiac anomaly which is not accompanied by other cardiac abnormalities, with the exception of two cases. We report a case of a 33-year-old male patient with isolated LV apical hypoplasia combined with infundibular pulmonary stenosis and aortic stenosis. We review a literature focusing on the characteristic magnetic resonance features and combined cardiac abnormalities.
Adult ; Aortic Valve Stenosis/*complications/diagnosis ; Diagnosis, Differential ; Echocardiography ; Electrocardiography ; Humans ; Hypoplastic Left Heart Syndrome/*complications/diagnosis ; Magnetic Resonance Imaging, Cine ; Male ; Pulmonary Valve Stenosis/*complications/diagnosis

BACKGROUND: Twelve patients with acyanotic tetralogy of Fallot (TOF), characterized by the combination of a malaligned ventricular septal defect (VSD) and infundibular pulmonic stenosis with the clinical finding of acyanosis at rest, underwent surgical correction between January 1988 and July 1997. MATERIALS AND METHODS: 9.92% of patients with the diagnosis of TOF were acyanotic TOF in the same period. Ages ranged from 12 to 42 months (mean 25.2 months). 2D-echocardiographic studies, cardiac catheterization, and angiocardiograms were performed in all patients before operation. The preoperative mean systemic arterial oxygen saturation was 93.5%. According to the 2D-echocardiographic analysis, there was Lt-to-Rt shunt through VSD in 4 patients, bidirectional shunt in 2 patients, and no shunt in 6 patients. RESULTS: The preoperative mean right ventricle to pulmonary artery (RV-PA) pressure gradients were 52.3 mmHg on 2D-echocardiogram and 48.4 mmHg on cardiac catheterization. The repair of ventricular septal defect was performed through a right atrial approach and the hypertrophic infundibular muscle bundles were resected by the transatrial and transpulmonary approach. Six patients (50%) received a transannular patch. The mean cardiopulmonary bypass time was 135.0 minutes, and the aortic crossclamp time was 87.8 minutes. Postoperative complications included bleeding necessitating reentry in one and chylothorax in one. No patient died after operation and there were no late deaths. Postoperative 2D-echocardiograms revealed tiny patch dehiscence in 5 cases and a moderate RV-PA pressure gradients (mean 15.3 mmHg). All patients were in New York Heart Association functional class 1 after operation. CONCLUSIONS: acyanotic TOF is the uncommon form of TOF, and acyanotic TOF can be repaired with a good outcome.
Cardiac Catheterization ; Cardiac Catheters ; Cardiopulmonary Bypass ; Chylothorax ; Diagnosis ; Heart ; Heart Septal Defects, Ventricular ; Heart Ventricles ; Hemorrhage ; Humans ; Oxygen ; Postoperative Complications ; Pulmonary Artery ; Pulmonary Valve Stenosis ; Tetralogy of Fallot*

No abstract available.
Anti-Bacterial Agents/therapeutic use ; Biopsy ; Echocardiography, Doppler, Color ; Echocardiography, Transesophageal ; Endocarditis, Bacterial/complications/diagnosis/drug therapy/*microbiology ; Fluorescent Antibody Technique ; Heart Septal Defects, Ventricular/*complications/diagnosis/surgery ; Humans ; Male ; Middle Aged ; Predictive Value of Tests ; Pulmonary Valve Stenosis/*complications/diagnosis ; Purpura, Schoenlein-Henoch/diagnosis/drug therapy/*etiology ; Risk Factors