(1977). Thorax, 32,[365][366][367][368][369] Syndrome of congenital ventricular diverticulum and midline thoraco-abdominal defects. The long-term follow-up of a 30-year-old patient who had a correction of the syndrome of left ventricular diverticulum and thoraco-abdominal defects is presented. The main features of the syndrome include a diverticulum of the left ventricle, a ventricular septal defect, and sometimes other cardiac anomalies. The thoraco-abdominal defects consist of foreshortened sternum, pericardial and diaphragmatic defects, and umbilical hernia. The association of these anomalies is thought to be due to a developmental failure of the primitive paramidline mesoderm.The diagnosis can be made clinically by the presence of a pulsatile, epigastric mass associated with signs of cardiac septal defects and dextrocardia. The prognosis of patients with this syndrome depends mainly on the associated abnormalities although rupture of the diverticulum can be a fatal complication.The treatment of choice is resection of the diverticulum combined with repair of associated anomalies which can give good early and late results.The association of a congenital ventricular diverticulum with -other cardiac abnormalities and midline thoraco-abdominal defects has been described by Cantrell et al. (1958). Its recognition is essential for accurate identification of the different anomalies and their proper treatment. The purpose of this paper is to describe the long-term result in a patient in whom complete correction of the syndrome had been performed six years previously and to draw attention to the characteristic lesions and their significance.
Case reportA 30-year-old woman was admitted to Harefield Hospital with a history of cyanosis and increasing dyspnoea which were first noted when she was 4 years old after an operation for umbilical hernia. There was no family history of cardiac disease or congenital anomalies. On admission the physical signs included clubbing of the fingers and moderate cyanosis. The jugular venous pressure was normal and the radial pulse regular at a rate of 90 per minute. The blood pressure was 120/80 mmHg. There was also a slight prominence of the right chest with no definite evidence of ventricular hypertrophy. A pulmonary systolic thrill was felt and a loud systolic murmur was heard in the right parasternal area over the fourth interspace. In the abdomen there was the scar of an umbilical herniorrhaphy but no masses were palpable.Laboratory tests showed a red cell count of 4-3 X 1012/1 with a haemoglobin of 18 g/dl and haematocrit of 44%. Serological tests for syphilis were negative. The radiograph showed oligaemic lung fields and the heart was slightly enlarged and situated to the right of the spine with the appearance of dextrocardia. However, the aortic knuckle and the stomach were on the left (Fig. 1)
