Syndrome of single ventricle without pulmonary stenosis but with left atrioventricular valve atresia and interatrial obstruction

Rao, P. Syamasundar; Kulangara, Raju J.; Moore, H. Victor; Strong, William B.

doi:10.1016/s0022-5223(19)37671-8

Cited by 25 publications

(10 citation statements)

References 15 publications

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Section: Management Strategies To Address the Single Ventricle Defectsmentioning

confidence: 99%

“…In babies with single ventricle and mitral atresia, inter-atrial obstruction is frequently present [ 62 , 63 ]. In such infants, predictable fall in pulmonary vascular resistance (PVR) takes place after relief of atrial obstruction either by balloon septostomy or surgery [ 63 ]. Therefore, banding of the PA should be performed without hesitation at the time of alleviating the atrial septal obstruction in order to reduce the probability for CHF, reduce the PVR and PA pressure, prevent pulmonary vascular obstructive disease (PVOD), and pave the way for Fontan circulation [ 62 , 63 ].…”

Section: Management Strategies To Address the Single Ventricle Defectsmentioning

confidence: 99%

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Single Ventricle—A Comprehensive Review

Rao

2021

Children

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In this paper, the author enumerates cardiac defects with a functionally single ventricle, summarizes single ventricle physiology, presents a summary of management strategies to address the single ventricle defects, goes over the steps of staged total cavo-pulmonary connection, cites the prevalence of inter-stage mortality, names the causes of inter-stage mortality, discusses strategies to address the inter-stage mortality, reviews post-Fontan issues, and introduces alternative approaches to Fontan circulation.

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Section: Management Strategies To Address the Single Ventricle Defectsmentioning

confidence: 99%

Section: Management Strategies To Address the Single Ventricle Defectsmentioning

confidence: 99%

Single Ventricle—A Comprehensive Review

Rao

2021

Children

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“…The medical and surgical treatment of other CHDs, namely, hypoplastic left heart syndrome [72,73,81], pulmonary atresia with intact ventricular septum [65,69,72,73,81,82], double-outlet right ventricle [65,76,83], univentricular hearts including double-inlet left ventricle [17,34,65,72,76], corrected transposition of the great arteries [65,[84][85][86]], Ebstein's anomaly of the tricuspid valve [65,85,[87][88][89], pulmonary atresia without intact ventricular septum (with VSD) [17,34,65,72,76,[84][85][86], mitral atresia with normal aortic root [34,65,90,91], interrupted aortic arch [65,92], and dextrocardia and heterotaxy syndromes (asplenia and polysplenia) [65,[93][94][95][96] was also discussed. The presently used medical, trans-catheter and surgical procedur...…”

Section: Ecg: Electrocardiogrammentioning

confidence: 99%

Evolution of Pediatric Cardiology over the Last 50 Years - Part IV

Syamasundar¹

2021

Int J Clin Cardiol

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Check for updatesphysiologically advantageous VSDs occurs as often as in isolated VSDs. The mechanisms of closure is also similar to that seen in isolated VSDs. VSD closures were documented to take place in utero, during infancy, childhood, and adolescence and continue through adulthood. It was concluded that there is a great natural tendency for spontaneous closure of VSDs whether it be an isolated defect or is a component of a complex heart defect [10][11][12][13][14][15][16].Spontaneous closure of VSD in children with tricuspid atresia and normally related great arteries (Type I) produces decreased pulmonary blood flow and requires surgical intervention earlier than otherwise needed. We recommended that a Blalock-Taussig (BT) shunt (preferably on the left side) is carried out instead of a Glenn anastomosis (the concept of bidirectional Glenn has not yet been crystallized as of that time) because VSD closure following Glenn will result in no blood flow into the left pulmonary artery (Figure 3). If further palliation is required prior to Fontan procedure, a BT shunt on the right side, Glenn anastomosis, or enlargement of the VSD may be entertained. In subjects with tricuspid atresia and transposition of the great arteries (Type II), VSD closure produces left ventricular outflow obstruction. Therefore, it is highly important that the size of the VSD is appraised before planning for Fontan. If the VSD is small, enlarging the VSD surgically or bypassing the VSD and the right ventricle via a pulmonary artery-to-ascending aorta anastomosis (Damus-Kaye-Stansel) or by a left ventricle-descending aorta conduit (Figure 4) should be considered [10][11][12][13][14]. In addition to tricuspid atresia and double outlet right

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“…Originally described in 1966 by Rashkind, 1 balloon atrial septostomy (BAS) has been extensively used in palliation of transposition of the great arteries [2][3][4][5][6][7] and other congenital heart defects, namely, tricuspid atresia, 8,9 pulmonary atresia with intact ventricular septum, 10,11 mitral atresia, 12,13 and total anomalous pulmonary venous connection with interatrial obstruction. [14][15][16] The original concept and subsequent success of BAS are based on the fact that the lower margin of the patent foramen ovale (PFO) is very thin and membranous and can be torn by forcefully withdrawing a balloon filled with fluid, usually diluted contrast material, from the left atrium (LA) to the right atrium (RA).…”

Section: Introductionmentioning

confidence: 99%

Atrial Septostomy without Thoracotomy: The Experience with Transcatheter Knife Atrial Septostomy

Rao

Mardini

1983

Ann Saudi Med

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Four infants, three with transposition of the great arteries and one with mitral atresia and single ventricle, who did not improve following balloon atrial septostomy are presented. Each patient underwent transcatheter knife atrial septostomy with resultant improvement of interatrial mixing (in the transposition patients, there was immediate improvement in two and delayed improvement in one patient) or interatrial obstruction (in the mitral atresia patient). It is suggested that knife atrial septostomy is a safe and effective technique for nonsurgical palliation of congenital heart defects to enlarge a restrictive interatrial communication if balloon atrial septostomy has not been successful.

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Syndrome of single ventricle without pulmonary stenosis but with left atrioventricular valve atresia and interatrial obstruction

Cited by 25 publications

References 15 publications

Single Ventricle—A Comprehensive Review

Single Ventricle—A Comprehensive Review

Evolution of Pediatric Cardiology over the Last 50 Years - Part IV

Atrial Septostomy without Thoracotomy: The Experience with Transcatheter Knife Atrial Septostomy

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