Ten cases of total abnormal pulmonary venous connection are described: 5 cases with supracardiac drainage, 4 with sub-diaphragmatic drainage and one with intracardiac drainage, 3 cases with supradiaphragmatic drainage, and 4 with subdiaphragmatic drainage have undergone surgical treatment. Some developmental, embryological and pathophysiological signs of this malformation are reported. The more significant clinical and instrumental data arising from our personal cases and from review data are discussed. In the clinical description a distinction is made between cases with venous obstruction and those without venous obstruction. The importance of hemodinamic tests, which allow us to identify the type of connection present, and whether or not there are associated malformations, are underlined. The natural history is conditioned by the presence of venous obstruction, by the value of the pulmonary hyper-flow and by the possibility of communication between the two circulatory districts. The prognosis of the cases with marked obstruction, and of those with irreversible cardiac failure is one of rapid deterioration: surgical correction in the first months of life is the only therapeutic possibility, even if the death risk is high. The cases without venous obstruction, and with fairly normal weight and circulation, are corrected surgically at a later age with much more favourable results.

Child↗

PubMed1978

[Left ventricular-right atrial communication. Diagnostic and therapeutic aspects: report of 16 cases (author's transl)].

16 cases of left ventricular-right atrial communication are reported. The most consistent clinical features, suggestive of this condition, were: auscultatory findings of V.S.D., electrocardiographic patterns of right atrial enlargement and biventricular hypertrophy, considerable right atrial enlargement coexisting with signs of left-sided cardiac enlargement and increased polmonary vascularity to Xray examination, echocardiographic fluttering of the tricuspid valve. Cardiac catheterization revealed an increased oxygen content in the right atrial or ventricular blood without the passage of the catheter through an A.S.D. The definitive diagnosis was established by left ventricular angiocardiography which demonstrated immediate opacification of a dilated right atrium through a septal defect; the location of the septal defect in the cardiac septum was attempted. In patients who suffered from a large left to right shunt were recommended for surgical treatment also in view of the relatively bad tolerance of this defect and low operative risk. There were two deaths: one baby died of early congestive failure. 9 cases were operated upon with one immediate postoperative death. The remaining, controlled by clinic follow-up, had good results.

Adult↗

PubMed1977