Unusual Combination of Hypoplastic Left Ventricle, Atrioventricular Septal Defect With Restrictive Ventricular Septal Defect, and Common Arterial Trunk

Tripathi, Ravi Ranjan; Sridhar, Anuradha; Chidambaram, Shanthi

doi:10.1177/2150135111434165

Cited by 4 publications

(6 citation statements)

References 5 publications

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“…I read with interest the report by Tripathi and colleagues 1 concerning their experience with patients having the combination of an atrioventricular septal defect and a common arterial trunk, the common trunk arising exclusively from the morphologically right ventricle. The authors suggest that this combination of common atrioventricular and ventriculoarterial junctions has not previously been reported.…”

Section: Common Arterial Trunk With Restrictive Ventricular Septal Dementioning

confidence: 99%

“…Indeed, since normal fusion proceeds in distal to proximal direction, it is highly unlikely that there could be independent fusion of the proximal cushions. It is equally unlikely that development could proceed as suggested by Tripathi and colleagues 1 to account for common arterial trunk with intact ventricular septum. It is, of course, possible to find this combination when the common trunk arises exclusively from the right ventricle.…”

mentioning

confidence: 96%

“…Much has been revealed over the past three decades concerning separation of the intrapericardial pulmonary and aortic channels. Tripathi and colleagues 1 rightly stress the importance of migration of cells from the neural crest in normal development, although they do not provide a reference to support this statement. It was Kirby and her colleagues who placed emphasis on this important feature.…”

mentioning

confidence: 97%

“…It is, of course, possible to find this combination when the common trunk arises exclusively from the right ventricle. As Tripathi and colleagues 1 suggest, this is equivalent to double outlet right ventricle with intact ventricular septum. These lesions, however, are the consequence of subsequent closure of a preexisting interventricular communication.…”

mentioning

confidence: 97%

“…It is, of necessity, absent in the setting of common arterial trunk, which is due to failure of septation of the developing outflow tract. 4 When offering their account of morphogenesis, Tripathi and colleagues 1 place great emphasis on the review provided by Carr and colleagues. 5 The group working in Chicago described a case in which the truncal valvar leaflets closed on the crest of the muscular ventricular septum during ventricular diastole.…”

mentioning

confidence: 99%

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Common Arterial Trunk With Restrictive Ventricular Septal Defect

Anderson

2013

World J Pediatr Congenit Heart Surg

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Section: Common Arterial Trunk With Restrictive Ventricular Septal Dementioning

confidence: 99%

mentioning

confidence: 96%

mentioning

confidence: 97%

mentioning

confidence: 97%

mentioning

confidence: 99%

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Common Arterial Trunk With Restrictive Ventricular Septal Defect

Anderson

2013

World J Pediatr Congenit Heart Surg

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Common arterial trunk in functionally univentricular hearts: a case series

et al. 2022

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Introduction: The association of a univentricular heart defect with common arterial trunk is extremely rare. There is a lack of population-based outcome studies reported in the literature. Methods: The hospital records, echocardiographic and other imaging modality data, outpatients’ records, operation notes, and other electronic data were reviewed. Patients were reviewed, and the final outcomes of surgery were observed. Results: Six cases (two males) with common arterial trunk presented over a 30-year period. Five had a complete unbalanced atrioventricular septal defect (83%) and one (17%) had tricuspid atresia associated with common arterial trunk. All had antenatal diagnosis. Two cases (33%) were excluded from initial surgical palliation due to Trisomy 21 in one and severe truncal valve regurgitation in one. Initial surgical palliation was performed in four cases (67%) at median age of 31 days (2–60) and consisted of disconnection and reconstruction of the pulmonary arteries and establishing controlled pulmonary blood flow. There were no early deaths. Conversion to cavopulmonary shunt was not possible in two due to severe airway problems in one and pulmonary arteries anatomy in one. They died at 11 and 16 months, respectively. Two patients (33%) underwent cavopulmonary shunt with 1 (17%) being alive at 18 months – 12 months after cavopulmonary shunt. The second patient proceeded to Fontan completion at 19 months but required catheter takedown 3 months later and died 3.5 years later. Conclusions: Univentricular hearts with common arterial trunk carry extremely poor short- to medium-term outcomes. This should inform antenatal and postnatal counselling and decision-making.

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Anomalous Fusion of Right Pulmonary Artery to Aortic Arch: Case Report of a Rare and Fatal Congenital Malformation in a Newborn and a Literature Review

et al. 2018

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Patient: Female, newbornFinal Diagnosis: Anomalous fusion of right pulmonary artery-to-aortic archSymptoms: Respiratory failureMedication: —Clinical Procedure: —Specialty: CardiologyObjective:Congenital defects/diseasesBackground:We present a report of a rare cardiac malformation case as well as a review of the literature. In addition, the diagnostic features are discussed.Case Report:The case of a female newborn who died on her third day of life was studied at the Institute of Legal Medicine, University of Chieti-Pescara (Italy). The investigations around her death revealed a cardiac congenital malformation, seen as a rare variant of a common arterial trunk, in which the aorta was fused with the right branch of the pulmonary artery. The ascending aorta showed hypoplasia, while the coronary arteries were free of any pathological findings. The atrial septum showed a closed foramen ovale and the ventricular septum did not show any defect. Only an isolated right ventricular hypertrophy and dilation with no other cardiac abnormalities was found. The cause of death was acute respiratory failure on the third day of extrauterine life when the ductus Botalli closed. The karyotype analysis performed in this case was normal, and the fluorescent in situ hybridization analysis did not show the 22q11.2 microdeletion suggestive of the DiGeorge syndrome.Conclusions:These findings underline the value of 3-dimensional/4-dimensional ultrasound imaging when added to a fetal cardiology screening program, and the need for improvements in postnatal screening routines by using pulse oximetry in order to discover isolated vascular defects before circulatory collapse occurs, as well as to reduce the medico-legal disputes in cases of missed diagnosis. We found the relevant literature search lacked a description of this congenital malformation, which supports our deeper perinatal investigation.

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Unusual Combination of Hypoplastic Left Ventricle, Atrioventricular Septal Defect With Restrictive Ventricular Septal Defect, and Common Arterial Trunk

Cited by 4 publications

References 5 publications

Common Arterial Trunk With Restrictive Ventricular Septal Defect

Common Arterial Trunk With Restrictive Ventricular Septal Defect

Common arterial trunk in functionally univentricular hearts: a case series

Anomalous Fusion of Right Pulmonary Artery to Aortic Arch: Case Report of a Rare and Fatal Congenital Malformation in a Newborn and a Literature Review

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