Variant of tetralogy of fallot with absent pulmonary valve leaflets and origin of one pulmonary artery from the ascending aorta

Calder, A. Louise; Brandt, P. W. T.; Barratt‐Boyes, B. G.; Neutze, John M.

doi:10.1016/0002-9149(80)90612-8

Cited by 67 publications

(21 citation statements)

References 78 publications

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“…Patients with A A V cannot survive beyond the newborn stage due to serious heart failure, which is in sharp contrast to APV patients, whose median age at death is 6.5 months [28]. The fetal cases in our study indicated that the inception of AAV could go as far back as midgestation.…”

Section: Discussionmentioning

confidence: 59%

Absent aortic and pulmonary valves: Investigation of three fetal cases with cystic hygroma and review of the literature

et al. 1994

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Absent semilunar valve was found in three fetal cases with cystic hygroma. Two cases which simultaneously showed absent aortic and pulmonary valves (AAV and APV, respectively) had double-outlet right ventricle. The third case, which lacked only the aortic valve, had atrioventricular septal defect and anomalous origin of the right subclavian artery. Two of the three cases had a markedly hypoplastic thymus. Fifteen AAV and 179 APV cases, including the above-mentioned cases and others reported elsewhere, were discussed with special reference to the pathogenesis of absent semilunar valve. Of the 15 AAV cases, hypoplasia of the left heart was observed in 11 cases (73.3%), double-outlet right ventricle in 5 (33.3%), and aortic arch malformations in 6 (40.0%). In the 179 APV cases, there were 111 tetralogy of Fallot (62.0%) and 44 right-sided aortic arch (24.6%). DiGeorge anomaly was found in one AAV and eight APV cases. These results indicate a pathogenesis that is possibly related to hemodynamic abnormalities or abnormal neural crest cells. Further investigation will be needed to elucidate a more definite pathogenesis of absent semilunar valve.

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Section: Discussionmentioning

confidence: 59%

Absent aortic and pulmonary valves: Investigation of three fetal cases with cystic hygroma and review of the literature

et al. 1994

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“…Since then, there have been several reports (Kirklin et al 1965 ; Kawada et al 1971;Imai et al 1974 ;Calder et al 1980;Robita 1985) of surgical correction of this anomaly. But, the operative results had not been satisfactory not only because complicated operations were required but also because this anomaly had some inherent problems.…”

Section: Resultsmentioning

confidence: 99%

Tetralogy of Fallot with Anomalous Origin of Left Pulmonary Artery.

Endo

Haneda

Mohri

et al. 1992

Tohoku J. Exp. Med.

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patients of tetralogy of Fallot with anomalous origin of left pulmonary artery underwent intracardiac repairs at Tohoku University Hospital. They were 2 males and 5 females with ages ranging from 4 to 26 years old. The right pulmonary artery connected to right ventricle in all cases, whereas no communications between right ventricle and the left pulmonary artery were found. The left pulmonary artery directly originated from the ascending aorta in 2 patients (group I) and connected to the ductus arteriosus in 5 patients (groups II and III). In 2 patients (group II), the left pulmonary artery was separated from the pulmonary arterial trunk by the intraluminal membrane, receiving blood supply through the ductus. In the remaining 3 patients (group III), there were no continuations between both pulmonary arteries. At the correction, communication between the left pulmonary artery and the pulmonary arterial trunk could be reconstructed in groups I and II. However, it was not possible in group III, because the ductus arteriosus and the left pulmonary artery had already been occluded before the intracardiac repair. In group III, ventricular septal defect was closed using a one-way valved patch or a perforated patch to decrease supersystemic right ventricular pressure. Postoperative right ventricular aortic pressure ratio was between 0.5 and 0.8 in groups I and II, and between 0.8 and 1.0 in group III. Three patients (one in each group) died after the operation. Severe pulmonary vascular obstructive disease was found in the left lung of group I. Tetralogy of Fallot with anomalous origin of unilateral pulmonary artery should be corrected before the advance of pulmonary vascular obstructive disease in case pulmonary artery originates from the ascending aorta, and before the occlusion of the ductus arteriosus in case pulmonary artery originates from it.Tetralogy of Fallot ; anomalous origin of pulmonary artery ; correctionThe results of the correction of Tetralogy of Fallot have been improved. However, Tetralogy of Fallot complicated with other anomalies still has some difficulties for total correction. Among various anomalies, the anomalous origin of left pulmonary artery was encountered 7 (3%) out of 222 cases of Tetralogy of Fallot during past 20 years of our experience. These cases were classified into 3

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“…to the development of distal pulmonary arteries that surround the inner lung branches of the bronchial tree and subsequently the compression and underdevelopment of the cartilaginous apparatus causing the emphysema 10 . In the early '80s and '90s as well as in the early 2000s, it was believed that the later surgery in the infancy, the higher the odds of a good postoperative prognosis in patients with APVS 11,12 . Due to the fact that this is a very rare defect, the literature available so far has a small database of postoperative results, and in the last decade there were only six reports comprehensive publications based on the analysis of a dozen cases (12 -52) [13][14][15][16][17][18] .…”

confidence: 99%

Absent Pulmonary Valve Syndrome – Special Prenatal Care and Early Surgery in Obstetric/Cardiac Center - A New Hope for Postnatal Survivors?

Zych-Krekora

Krekora

Kopala

et al. 2017

Prenatal Cardiology

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Introduction: APVS (Absent Pulmonary Valve Syndrome) is a rare congenital heart disease. Its incidence according to The Polish National Registry of Fetal Cardiac Pathology in years 2004 - 2016 was 0.6%.This disease is caused by the absence or the residual pulmonary artery valve resulting in significant dilation of the pulmonary trunk and its branches. In utero deaths are reported. After birth the major problem is respiratory failure and high preoperative and postoperative mortality. Material and methods: In 1995 to 2016, 11 fetuses with APVS were diagnosed in our unit, at the average 27,5 weeks of gestation (min. 18.5- max 37.1 weeks of gestation). Two groups were analysed in this series of cases: “Old one” by 2011 (n = 6) and “New one” since 2011 (n = 5) and perinatal care as well as survival were compared. We analysed the fetal echo results, perinatal care including transplacental digoxin and steroids treatment in NEW group, the longevity of the pregnancy and neonatal/infants outcome. Results: In Old group the average day of cardiac surgery was day 91st after birth (max. 161) and the survival was 50% . In the New group the average surgery day was 41st day and the postoperative survival was 60%, however there was no statistical significance ( p > 0,05). Conclusions: There is no single parameter from prenatal life in foetuses with APVS which may allow to predict the positive outcome meaning neonatal survival. However optimal perinatal care (early detection of defect, transplacental digoxin at least 3-4 weeks, steroids, no preterm delivery, on-time delivery, postnatal care in tertiary center) and relatively early cardiac surgery may have combined impact on the improvement of survival after prenatal diagnosis of APVS, however more data are necessary to prove this hypothesis.

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Variant of tetralogy of fallot with absent pulmonary valve leaflets and origin of one pulmonary artery from the ascending aorta

Cited by 67 publications

References 78 publications

Absent aortic and pulmonary valves: Investigation of three fetal cases with cystic hygroma and review of the literature

Absent aortic and pulmonary valves: Investigation of three fetal cases with cystic hygroma and review of the literature

Tetralogy of Fallot with Anomalous Origin of Left Pulmonary Artery.

Absent Pulmonary Valve Syndrome – Special Prenatal Care and Early Surgery in Obstetric/Cardiac Center - A New Hope for Postnatal Survivors?

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