Hemianomalous pulmonary venous drainage with intact atrial septum is a rare congenital anomaly and reports of its surgical repair and the long-term complications related to the correction are only infrequently encountered in the literature. We report the case of a patient with hemianomalous pulmonary venous drainage and intact atrial septum who underwent surgical repair using a pericardial baffle and creation of an “atrial septal defect” aged 15 years. Dyspnoea and recurrent chest infections started 7 months after surgery when he was seen by a respiratory physician without cardiac followup. He presented again aged 28 years with a recurrent pneumonia investigated over 6 weeks and heart pronounced normal from examination and echocardiography. Correct diagnosis was made in Grown Up Congenital Heart (GUCH) clinic stimulating review of data and catheterisation with pulmonary artery angiography which confirmed it. We feel that this case highlights the importance of specialist care and followup for GUCH patients.
A gentleman born in 1979 had an asymptomatic murmur during childhood. Investigations confirmed hemianomalous pulmonary venous drainage to the right atrium without an atrial septal defect. The parents were Jehovah’s witnesses and refused cardiac surgery with use of blood products. When aged 15 years, an adult cardiologist advised surgery and the patient was operated on by a general cardiac surgeon unused to congenital heart disease but willing not to use blood. The atrial septum was confirmed to be intact at operation and the oval fossa was enlarged to allow redirection of blood from the right pulmonary veins using a baffle of autologous pericardium to the left atrium. Recovery was uncomplicated. Seven months later he complained of increasing dyspnoea on effort and chest infections. He was reviewed once by a respiratory physician without further investigations or referral to cardiology. He trained and worked as a stonemason.
He presented again aged 28 years, with a three-week history of right-sided pleuritic chest pain, high fever and a dry cough. On examination there was reduced air entry, coarse crepitations and a pleural rub at the right base. Chest radiography showed a dense patchy consolidation of the right lower lobe, probably also involving the middle lobe (Figure
Chest radiographs (a) at presentation in GUCH clinic and (b) after several weeks of antibiotic treatment. Right pulmonary venous obstruction is suggested by the presence of dilated venules, Kerley lines and fluid in the fissures, all more obvious after the consolidation was treated.
Despite the “normality’’ of the heart but because of past cardiac surgery, he was referred to the monthly Grown Up Congenital Heart (GUCH) clinic. A diagnosis of obstructed right pulmonary venous drainage was made from the chest X-ray (Figure
Contrast computerized tomography scan of the thorax showing right pulmonary venous obstruction (arrow), right lung consolidation and posterior pleural effusion.
A cardiac catheter study further confirmed no forward flow to the right lung at pulmonary artery wedge angiography, with normal flow to superior caval vein and left pulmonary artery. Pulmonary artery systolic pressure was 26 mmHg. After retrograde catheterisation of the left atrium, a baffle stump was seen but was found to be blind-ending and could not be crossed. Discussion of management with the GUCH Unit team at The Heart Hospital London concluded that it was unsuitable for intervention or operative reconstruction. The only treatment recommended was right pneumonectomy. The patient and family adamantly refused operation with use of blood and no surgeon agreed to perform it without blood as it is likely to be a very vascular operation. The patient has not had further infective recurrences but still complains of exertional dyspnoea. The family is searching for a surgeon who accepts their conditions.
Partial anomalous pulmonary venous drainage with associated atrial septal defect is not an uncommon congenital malformation [
This case demonstrates points of clinical importance. The general cardiac surgeon who undertook the repair may not have been familiar with such a rare anomaly. Pulmonary vein obstruction is a known complication when pericardium is used in this way [
This patient highlights the importance of having GUCH patients seen by specialists familiar with congenital heart problems [
The authors would like to thank Dr. Joseph DeGiovanni (Senior Paediatric Cardiologist and Interventionalist Birmingham Children’s Hospital, UK) who performed the diagnostic catheter study and Dr. Oscar Aquilina (Consultant Cardiologist and Electrophysiologist Mater Dei Hospital, Malta) who is part of the GUCH team in Malta.