Unusual interatrial communication after the Fontan procedure

Westerman, G.R.; Readinger, Richard I.; Devanter, Stephen H. Van

doi:10.1016/s0022-5223(19)38580-0

Cited by 6 publications

(3 citation statements)

References 13 publications

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“…26 At the cardiac level, a wholly or partially unroofed coronary sinus, and/or anomalous connections of one or more cardiac veins connecting to the pulmonary venous atrium, will contribute to systemic desaturation. [104][105][106][107][108] These peculiar connections between the right atrium or coronary sinus with the left atrium have necessitated a better understanding of the normal venoatrial connections, 109 and the nature of the myocardial connections between left atrial myocardium and the muscular walls of the coronary sinus. 110 Partial hepatic venous exclusion was advocated to optimize postoperative Fontan haemodynamics.…”

Section: "Mimics" Of Pulmonary Arteriovenous Fistulasmentioning

confidence: 99%

The biological “scrabble” of pulmonary arteriovenous malformations: considerations in the setting of cavopulmonary surgery

2004

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Pulmonary arteriovenous fistulas are vascular malformations, which, by virtue of producing abnormal vascular connections proximal to the units of gas exchange, result in intrapulmonary right-to-left shunting. These malformations or fistulas reflect at least in part disordered angiogenesis, and less commonly recruitment and dilation of pre-existing vascular channels. Pulmonary arteriovenous fistulas occur in a number of diverse clinical settings. Such fistulas are a well-established feature of the Weber-Osler-Rendu complex, or hereditary haemorrhagic telangiectasia, an autosomal dominant vascular dysplasia characterized by mucocutaneous telangiectasis, epistaxis, gastrointestinal haemorrhage, and arteriovenous malformations in the lung, brain, liver and elsewhere. They are also seen in the patient with acute or chronic liver disease, disease that is usually but not invariably severe, or those with non-cirrhotic portal hypertension. They may occur as congenital malformations, single or diffuse, large or small in isolation, and when large or extensive enough may result in hypoxaemia, clinical cyanosis, and heart failure. Cerebral vascular accidents are also a well-known complication of this disorder. An extensive literature has accumulated with regard to the pulmonary arteriovenous fistulas seen in the setting of the Weber-Osler-Rendu complex, and there is considerable information on the genetics, basic biology, clinical findings, complications and therapeutic interventions of these malformations in the setting of this syndrome. These issues, however, are not the primary considerations of this review, although some aspects of this fascinating disorder will be discussed later. Rather the focus will be on pulmonary arteriovenous malformations that develop in the setting of cavopulmonary surgery, and their relationship to the pulmonary arteriovenous fistulas occurring in the hepatopulmonary syndrome. The complex tapestry of these overlapping and intersecting clinical observations will be unfolded in the light of their chronology.

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Section: "Mimics" Of Pulmonary Arteriovenous Fistulasmentioning

confidence: 99%

The biological “scrabble” of pulmonary arteriovenous malformations: considerations in the setting of cavopulmonary surgery

2004

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“…3 ' 5 Such communications have been noted in postmortem series but are infrequently described as a clinical cause of cyanosis. 6 Transcatheter closure of atrial communications are reported following the Fontan operation. In general, these reports are limited to closure of surgical fenestrations, 7 residual atrial septal defects and persistent communications between the superior caval vein and the right atrium after construction of a Glenn shunt.…”

Section: S Ystemic Desaturation After the Fontan Proce-mentioning

confidence: 99%

An unusual cause of cyanosis after the modified Fontan procedure—closure of venous communications between the coronary sinus and left atrium by transcatheter techniques

1994

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Communication of the coronary sinus with the left atrium is an unusual anomaly. Two cases of tricuspid atresia are described where systemic desaturation developed following a modified Fontan procedure. In both, investigation demonstrated a communication between the coronary sinus and the left atrium. Transcatheter closure of these communications was achieved with a ductal device in one case and coil embolization in the other. Following occlusion and elimination of the right-to-left shunt, both patients became fully saturated in room air.

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“…In six (8%) patients persistent oedema, gross hepatomegaly and ascites developed after they had been well for three months to seven years (mean [1][2][3][4][5][6][7][8] years) after the Fontan procedure. In all six patients the mean right atrial pressure was raised (14-27 mm Hg, mean 20 mm Hg) and there was no biochemical evidence of protein losing enteropathy.…”

Section: Chronic Fluid Retentionmentioning

confidence: 99%

Abnormal cardiac signs after Fontan type of operation: indicators of residua and sequelae.

Leung

Benson

Smallhorn

et al. 1989

Heart

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The best result is a patient without any cyanosis, no fluid retention, and a quiet heart that can maintain an adequate output at rest and during exercise. After a Fontan type operation, abnormal cardiac signs such as cyanosis, chronic fluid retention, an organic heart murmur or arrhythmia cause concern. We studied these abnormal cardiac signs to see what their relation is to the residua and sequelae of the Fontan type of operation.Requests for reprints to Professor Robert

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Unusual interatrial communication after the Fontan procedure

Cited by 6 publications

References 13 publications

The biological “scrabble” of pulmonary arteriovenous malformations: considerations in the setting of cavopulmonary surgery

The biological “scrabble” of pulmonary arteriovenous malformations: considerations in the setting of cavopulmonary surgery

An unusual cause of cyanosis after the modified Fontan procedure—closure of venous communications between the coronary sinus and left atrium by transcatheter techniques

Abnormal cardiac signs after Fontan type of operation: indicators of residua and sequelae.

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