Introduction: Univentricle comprises about 1% of all congenital heart defects and is said to be the most severe form. In the past, patients with single ventricle seldom reach adulthood. However, due to empowerment in knowledge, pediatric care and technology, more patients are being seen in the adult clinics. We report a rare case of single ventricle with common atrium, common atrioventricular valve, pulmonic stenosis, and malposed great arteries.
Clinical Presentation: J.J. is a 28-year-old female who was born term with poor cry. At 3 years old, she developed left hemiplegia after presenting with measles and convulsions. With 5 months of therapy, she was able to ambulate independently but with spastic paralysis of the left arm. In school, she can cope up with the requirements except for subjects necessitating increased physical activity. Presently, she usually complains of dyspnea after climbing 1 flight of stairs and walking 3 blocks on level ground. Cyanosis is noted whenever she cries or laughs for >1 minute or when she's tachypneic.
On consult, she is comfortable and ambulatory. Vital signs were as follows; BP 150/110 mmHg, HR 73 bpm, RR 20 cpm, T 370C. She has reddish palpebral conjunctiva, cyanotic lips, JVP of 8 cmH2O, and with vesicular breath sounds. The precordium is adynamic with apex beat at the 5th ICS L MCL, no heaves, no thrills, soft & single S1, single S2, no S3, no S4, normal rate, regular rhythm, 3/6 systolic ejection murmur at the 2nd ICS left parasternal border . Abdomen is flat and soft with palpable liver edge. There is nail clubbing with cyanotic nail beds. Pertinent neurologic examination includes a spastic left upper extremity.
Laboratory tests showed polycythemia and hypoxemia. There is cardiomegaly and decreased pulmonary vascularity on chest x-ray. 12L ECG also showed sinus rhythm, RAD, RAA, RVH, IRBBB with non-specific ST-T wave changes. TTE & TEE revealed situs solitus of both atria with a non-restrictive atrial septal defect, secundum type. The atria drain into a common atrio-ventricular valve. The single ventricle has LV morphology with mildly depressed systolic function. No rudimentary chamber was noted. Both the main pulmonary artery and the aorta originate from the single ventricle with the aorta located anterior and to the right of the MPA. All four pulmonary veins drain into the RA. The pulmonic valve is stenotic while the aortic valve is structurally normal.
The patient is on regular follow up at the out-patient clinic and she is currently maintained on warfarin, digoxin and enalapril.
Conclusion: Univentricular hearts with associated complex anomalies is rare and patients seldom survive to adulthood without any surgical intervention. Care for these patients for them to live a normal and longer life is the real challenge to the physician.