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Journal ArticleDOI

Total anomalous pulmonary venous connection: Report of 93 autopsied cases with emphasis on diagnostic and surgical considerations.

TL;DR: Total anomalous pulmonary venous connection (TAPVC) is failure of development of the common pulmonary vein, with consequent ersistence and enlargement of embryonic collaterals between the lungs and the systemic veins.
About: This article is published in American Heart Journal.The article was published on 1976-01-01. It has received 204 citations till now. The article focuses on the topics: Total anomalous pulmonary venous connection & Coronary sinus.
Citations
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Journal ArticleDOI
TL;DR: To facilitate the preoperative diagnosis and surgical management of visceral heterotaxy and asplenia, 72 postmortem cases were reviewed with particular attention focused on the systemic and pulmonary venous connections.

105 citations

Journal ArticleDOI
TL;DR: The boy: girl ratio was calculated for all live births with cardiac malformation in Bohemia from 1977–1984 and found that there were significantly more girls than boys with persistent ductus arteriosus.
Abstract: The boy: girl ratio was calculated for all live births with cardiac malformation in Bohemia (population 6.3 million) from 1977–1984. Complete coverage of all deceased children in Bohemia by necropsy and of all patients with heart disease by our center enabled us to collect reliable data. Of 4409 children born with a heart defect, there were 2296 boys and 2113 girls, a ratio of 1.09: 1. In the total population of 664,218 children born during the same period of time, the ratio was 1.06:1. A higher proportion of boys was found with double outlet right ventricle (2.68:1), hypoplastic left heart (2.25:1), transposition of the great arteries (2.11:1), aortic stenosis (1.95:1), pulmonary atresia (1.55:1), tricuspid atresia (1.45:1), coarctation of the aorta (1.30:1), and corrected transposition of the great arteries (1.25:1). There were significantly more girls than boys with persistent ductus arteriosus (1:1.66), Ebstein's anomaly of the tricuspid valve (1:1.57), truncus arteriosus (1:1.22), atrioventricular septal defect (1:1.17), and tetralogy of Fallot (1:1.12). The difference in sex prevalence in the remaining heart defects was less than 10%.

93 citations

Journal ArticleDOI
01 Jan 1977-Heart
TL;DR: In the older patients with the latter anomaly dilation of the pulmonary arteries and right ventricle suggested that a large left-to-right shunt had preceded the onset of obstruction to pulmonary venous return and that the more severe right ventricular and septal hypertrophy in these cases might be the result of a longer duration of pulmonary hypertension.
Abstract: Quantitative morphometric techniques have been applied to the injected and inflated lung and to the heart in 9 infants with total anomalous pulmonary venous return dying with obstruction to pulmonary venous return. In 5 (mean age at death 55 days) pge 20 days) to an infradiaphragmatic site. Structural changes were present in the pulmonary circulation in all patients, even in the youngest, an 8-day-old child. In both types of total anomalous pulmonary venous return increased arterial muscularity was severe, as shown by increase in wall thickness and by extension of muscle into smaller and more peripheral arteries than normal; these changes tended to increase with age. Where the pulmonary venous blood drained to a supradiaphragmatic site, the severity of arterial medial hypertrophy correlated inversely with the magnitude of the pulmonary: systemic flow ratio, increasing as the pulmonary blood flow fell. Vein wall thickness was increased and in all but the youngest child the veins were 'arterialised'. At the lung periphery the arteries and alveoli appeared to have multiplied normally. Arterial size varied according to whether pulmonary venous blood drained above or below the diaphragm; the diameter of pre- and intra-acinar arteries was increased only in cases where the pulmonary venous return drained to a supradiaphragmatic site, being normal when it drained to an infradiaphragmatic site. In the heart the left ventricle was of normal size in all but one case. Dilation and severe hypertrophy of the right ventricle and septum were present only in cases of drainage to a supradiaphragmatic site. In the older patients with the latter anomaly dilation of the pulmonary arteries and right ventricle suggested that a large left-to-right shunt had preceded the onset of obstruction to pulmonary venous return and that the more severe right ventricular and septal hypertrophy in these cases might be the result of a longer duration of pulmonary hypertension. In contrast, in total anomalous pulmonary venous return to an infradiaphragmatic site it appears that obstruction to pulmonary venous return develops soon after birth and prevents a large increase in pulmonary blood flow, and thus neither the pulmonary arteries nor the right ventricle become dilated. In infants with total anomalous pulmonary venous return and obstruction to pulmonary venous return, it is striking how rapidly the pulmonary circulation develops new muscle.

93 citations

Journal ArticleDOI
TL;DR: Although the number of TAPVR cases is small, this epidemiologic study identifies hypotheses that may be further explored in morphogenetic and epidemiology studies.
Abstract: In total anomalous pulmonary venous return (TAPVR), the intrapulmonary venous plexus has failed to connect to the left atrium, so that the pulmonary veins drain into right atrial tributaries, frequently resulting in early postnatal circulatory distress The Baltimore-Washington Infant Study (BWIS), a population-based exploratory case-control study of cardiovascular malformations (CVM), identified 41 cases of TAPVR during 1981-1987: 15% of all CVM (N = 2659), a regional prevalence of 68/100,000 live births Of the TAPVR infants, 68% were diagnosed as neonates, 88% had surgery, and 512% were alive at 1 year of age Noncardiac malformations were present in nine cases (22%); the male-female ratio was 078 (1823) Compared with a control group representative of the birth cohort (N = 2,801), more TAPVR patients had low birthweight (less than 2,500 g: 162% vs 69%, short gestational age (less than 38 weeks: 189% vs 93%), and intrauterine growth retardation (IUGR) (268% vs 58%) Sociodemographic findings were similar to those of controls, except that fewer TAPVR mothers received private pregnancy care (595% vs 714%) Family history revealed no other TAPVR-affected members, but a significant linear trend of increased risk was found over the ordered malformation categories (familial noncardiac, cardiac, both) Bivariate analysis of TAPVR and exposure in life-style, hobbies, and work showed possible associations for exposure to lead (OR 29; 99% confidence interval [CI]: 12, 72), painting/paint stripping (OR 33; 99% CI: 13, 84), lead soldering (OR 133; 99% CI: 18, 992), and pesticides (OR 27; 99% CI: 12, 64) Multivariate analysis suggested an interaction between pesticide exposure and family history and, thus, a possible familial susceptibility to environmental teratogens Although the number of TAPVR cases is small, this epidemiologic study identifies hypotheses that may be further explored in morphogenetic and epidemiology studies Total anomalous pulmonary venous return (TAPVR) constitutes a well-defined clinical entity in which the pulmonary veins fail to enter the left atrium and instead drain into the right atrium or its systemic venous tributaries (Neill, '56; Rowe et al, '81) During intrauterine life, the malformation does not compromise the fetal circulation, since the pulmonary arterial resistance is high and the patent foramen ovale provides easy access of right atrial blood to the left side of the heart At birth, however, the pulmonary vascular resistance begins to fall, and the presence of a severe hemodynamic disturbance becomes increasingly evident (Ferencz et al, '71)(ABSTRACT TRUNCATED AT 400 WORDS)

78 citations

Journal ArticleDOI
TL;DR: One patient required two subsequent reoperations for persistent pulmonary venous obstruction, and another patient had superior vena cava obstruction necessitating reoperation, resulting in a mean follow-up of 26 months.

62 citations

References
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Journal ArticleDOI
TL;DR: Thirty-three of 37 infants under 10 kg in weight, with correctable lesions, survived this procedure, including 25 aged 8 days to 12 months, and is believed to have wide application in the neonatal and infant group.
Abstract: In an attempt to achieve safe intracardiac surgery in severely ill babies with congenital heart defects, a technique of deep hypothermia with surface cooling and limited bypass has been used. Under halothane anesthesia, these infants were cooled to a nasopharyngeal temperature of 26 C on a circulating water blanket and the temperature was lowered further to 22 C by a short period of total body perfusion. After a period of circulatory arrest which averaged 48 minutes at 22 C, during which the intracardiac repair was carried out, rewarming to 32 C was achieved by 20 minutes of total body perfusion and final rewarming to 36 C by surface means. Thirty-three of 37 infants under 10 kg in weight, with correctable lesions, survived this procedure, including 25 aged 8 days to 12 months. Conditions corrected were transposition (13), tetralogy of Fallot (9), total anomalous pulmonary venous connection (6), ventricular septal defect (6), and atrioventricular canal (3). The technique gave ideal operating conditions an...

288 citations

Journal ArticleDOI
TL;DR: The author has shown that early splenic primordia exist at the time of fusion of the A‐V canal cushions and there is a primitive gut mesentery in the left aspect of the dorsal mesogastrium.
Abstract: Six embryos measuring 9–12 mm in greatest length have been shown by the writer to contain early splenic primordia in the left aspect of the dorsal mesogastrium. Their ovulation age is estimated to be 31–36 days. Temporal relations of splenic formation to conotruncus septation and division of the A-V canal have been discussed. This last is divided before septation of the conotruncus is completed. The author has shown that early splenic primordia exist at the time of fusion of the A-V canal cushions. At the same time there is initiation of pulmonary lobation, and there is a primitive gut mesentery.

239 citations

Journal ArticleDOI
TL;DR: The pathologic and embryologic findings strongly suggest that cor triatriatum results from entrapment of the left atrial ostium of the common pulmonary vein by tissue of the right horn of the sinus venosus from which septum primum develops, leading to failure of incorporation of theCommon pulmonary vein into theleft atrium during the fifth embryonic week.

213 citations

Journal ArticleDOI
TL;DR: The examination of serial sections of human embryos between 24 and 34 days and the use of plastic reconstructions showed that the common pulmonary vein develops as an outgrowth from the medial superior wall of the left auricle and unites with the angioblastic plexus of the developing lung bud.
Abstract: The examination of serial sections of human embryos between 24 and 34 days (3 to 11 mm) and the use of plastic reconstructions, showed that the common pulmonary vein develops as an outgrowth from the medial superior wall of the left auricle and unites with the angioblastic plexus of the developing lung bud. No evidence was found that the vein connects directly with the sinus venosus in the early stages, and later shifts in position as the atrial septum grows. Anomalous pulmonary venous drainage is classified in four main types, and theories of development are briefly discussed.

184 citations