Absent inferior vena cava, symmetrical liver, splenic agenesis, and situs inversus, and their embryology.

Campbell, Maurice; Deuchar, D C

doi:10.1136/hrt.29.2.268

Cited by 63 publications

(21 citation statements)

References 17 publications

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“…2). it is an isolated lesion, as in our patient, but rather it near ly always is associated with other serious cardiac anoma lies such as defects of the atrial septum and the atrioven tricular canal [3] (absent in our patient) and as much as 70-80% of patients with polysplenia [4], This abnormali ty is of clinical significance if only because of the technical problems it poses during catheterization via the femoral vein or cannulation for cardiopulmonary bypass. More over, inadvertent ligation of the azygos vein has caused death since it is the only major route of venous drainage from below the diaphragm [5], In a review of 18 cases of anomalous inferior vena cava with azygos drainage, 'coro nary sinus rhythm' (low atrial pacemaker) was present in 56%.…”

Section: Case Reportmentioning

confidence: 99%

Isolated Anomalous Inferior Vena cava with Azygos Drainage

Ramsaran

Filiberti²,

Spodick³

1995

Cardiology

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In a 79-year-old female with shortness of breath, catheterization via the femoral vein encountered difficulty entering the right heart; venography revealed anomalous drainage of the inferior vena cava via a dilated azygos vein permitting subsequent catheterization. Similar difficulties during catheterization merit consideration of this congenital anomaly, which is virtually always discovered in the pediatric population in association with other congenital anomalies. Its unique discovery at advanced age indicates its benignity as an isolated abnormality.

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Section: Case Reportmentioning

confidence: 99%

Isolated Anomalous Inferior Vena cava with Azygos Drainage

Ramsaran

Filiberti²,

Spodick³

1995

Cardiology

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“…The association of splenic anomalies with malpositions and malformations of other organ systems, in particular, the cardiovascular system, have been well documented in the past 20 years Schafer, 1952, 1955;Ivemark, I955;Putschar and Manion, I956;Ruttenberg et al, I964;Campbell and Deuchar, I967;Moller et al, I967;Landing et al, 197I;Van Mierop, Gessner, and Schiebler, I972;Freedom, I972). The complexity ofthe cardiac malformation determines the clinical course and prognosis and frequently precludes correction by surgical techniques.…”

mentioning

confidence: 98%

Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases withspecial reference to diagnosis and prognosis.

Rose¹,

Izukawa²,

Moës³

1975

Heart

359

160

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This reviw presents the cardiac and non-cardiac malformations in 6o cases with asplenia and polysplenia with special reference to distinguishing factors which may be helpful in the clinical recognition of these syndromes.The asplenia cases were predominantly male and presented with cyanosis. Theyfrequently had transposttion of the great arteries (72%) with pulmonary stenosis or atresia (88%) and total anomalous pulmonary venous drainage (72%). Deaths were caused by cardiacfailure and anoxia in 57 per cent of cases. Most of the patients died in thefirstyear of life (79%), but longer survival is possible in the asplenia syndrome.The polysplenia cases were predominantly female and survived longer. The characteristic clinical findings were the relatively more benign presenting signs and the leftward or superiorly orientated P wave axis on the electrocardiogram. Conotrumcal abnormalities were less comnon and total anomalous pulmonary venous drainage did not occur. On angiography the inferior vena caval drainage via the azygos system was clearly identified and this was present in all cases at surgery.Our study indicated that the cardiac anomalies in polysplenia were less severe than they were in asplenia and therefore the prognosis in the former syndrome is likely to be more favourable. Three families had two affected sibs but no single genetic factor could be identified. The aetiology of these syndromes remains undetermined.

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“…This suggests a common cause of the two conditions. Campbell and Deuchar [1967] proposed a defect in situs determination as responsible for the development of either multiple spleens or splenic agenesis. They also discuss how three other common findings, symmetric liver, absence of the hepatic portion of the IVC, and bilateral superior vena cavae, could be defects in situs determination.…”

Section: Discussionmentioning

confidence: 98%

Probable autosomal recessive inheritance of polysplenia, situs inversus and cardiac defects in an Amish family

1983

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We report on an Amish family with five individuals in two generations with complex congenital heart disease. Autopsy findings in one and clinical examination in the others support the diagnosis of polysplenia "syndrome." In a mouse model, this spectrum of situs abnormalities and cardiovascular defects shows recessive inheritance with homozygotes having either situs solitus or situs inversus or ambiguous situs. The parents of the four affected sibs are fourth cousins. We think that the father of these four children is an affected but clinically normal homozygote, that his deceased sister was an affected homozygote, and it seems likely that they too had consanguinous parents.

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Absent inferior vena cava, symmetrical liver, splenic agenesis, and situs inversus, and their embryology.

Cited by 63 publications

References 17 publications

Isolated Anomalous Inferior Vena cava with Azygos Drainage

Isolated Anomalous Inferior Vena cava with Azygos Drainage

Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases withspecial reference to diagnosis and prognosis.

Probable autosomal recessive inheritance of polysplenia, situs inversus and cardiac defects in an Amish family

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