Rare forms of isolation of the subclavian artery: echocardiographic diagnosis and surgical considerations

McElhinney, Doff B.; Silverman, Norman H.; Brook, Michael M.; Reddy, V. Mohan; Hanley, Frank L.

doi:10.1017/s1047951100006855

Cited by 28 publications

(37 citation statements)

References 17 publications

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“…In fact, the morphology of the atrioventricular junction is highly homogenous, as all patients except one from our series and the great majority of the previously published series (Gatzoulis et al, 1994;McElhinney et al, 1998;Schmid et al, 2000) presented a complete form of AVCD and a Rastelli type C (O'Blenes et al, 1998).…”

Section: Cardiac Phenotypementioning

confidence: 50%

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Genetic heterogeneity and phenotypic anomalies in children with atrioventricular canal defect and tetralogy of Fallot

Vergara

Digilio

Zorzi

et al. 2006

Clinical Dysmorphology

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Tetralogy of Fallot associated with the atrioventricular canal defect has been usually reported in association with Down syndrome. The aim of the present study was to describe the cardiac aspects and the genetic anomalies in children with this association of heart defects. We identified 64 patients with atrioventricular canal defect tetralogy of Fallot. All children underwent complete cardiovascular, clinical phenotypic and genetic evaluation. A genetic syndrome or extracardiac anomalies were found in 56 patients (87.5%). Down syndrome (43 patients, 67.2%) was the most frequent genetic diagnosis. Other syndromes were 8p deletion, trisomy 13, duplication 5p, cranio-cerebello-cardiac syndrome, Cantrell syndrome, CHARGE association, VACTERL association, and DiGeorge syndrome related to maternal diabetes. No patients in our series had 22q11 deletion. Tetralogy of Fallot with extreme dextroposition of the aorta was found in seven patients (only one with Down syndrome). Additional cardiac malformations were present in 23 patients (only 11 with Down syndrome). The association between atrioventricular canal defect and tetralogy of Fallot represents a cardiac phenotype with strong genetic characteristics. For this reason, a careful genetic examination is required. Our study confirms the high prevalence of Down syndrome, but also reveals a significant genetic heterogeneity. Additional cardiac defects are prevalent in patients without Down syndrome.

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Section: Cardiac Phenotypementioning

confidence: 50%

“…The surgical treatment of this association of congenital heart diseases is difficult and controversies still exist about the optimal approach (Gatzoulis et al, 1994;McElhinney et al, 1998;Schmid et al, 2000).…”

Section: Introductionmentioning

confidence: 99%

Genetic heterogeneity and phenotypic anomalies in children with atrioventricular canal defect and tetralogy of Fallot

Vergara

Digilio

Zorzi

et al. 2006

Clinical Dysmorphology

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show abstract

“…10) However, patients with cerebral insufficiency or claudication of the left arm may require reimplantation of the LSA into the aorta. In our patient, a Fontan type operation or total cavopulmonary connection at a later period may be the best option, along with reimplantation of the LSA into the aorta.…”

mentioning

confidence: 99%

Subclavian and Pulmonary Steal Phenomenon in Isolated Left Subclavian Artery with Left Lung Agenesis.

Chen

2002

Jpn Heart J

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SUMMARYIsolated left subclavian artery has been documented to be associated with several congenital cardiac defects, including tetralogy of Fallot and transposition of the great arteries. In some cases subclavian or pulmonary artery steal phenomenon may occur when the isolated left subclavian artery connects to the main pulmonary artery via a ductus arteriosus. The isolated left subclavian artery may be part of the cardiac manifestations in multiple congenital anomalies, such as VACTERL association. We describe subclavian and pulmonary steal phenomenon in a neonate with complex congenital heart disease, including an isolated left subclavian artery, and left lung agenesis. An isolated left subclavian artery is rare and its association with agenesis of the left lung has not been reported previously. CASE REPORTA male Taiwanese infant weighing 2760 grams was born at term to a G2P2 mother by vaginal delivery. The Apgar scores were 3 and 7 at one and five minutes, with a delay of initial crying for one minute. There was no family history of cardiac or skeletal anomalies. Physical examination at birth showed a slightly hypoplastic left hemithorax. The heart rate was 136 beats per minute, the respiratory rate 50 per minute, and blood pressure 45/25 mmHg and 62/34 mmHg in the left and right upper limbs, respectively. Systemic oxygen saturation was 80% on room air. The left brachial pulse was weak with a normal right brachial and femoral pulse. There was no cranial bruit. A grade 2/6 systolic ejection murmur was heard at the left midsternal border. Breath sounds were absent over the left chest. A chest x-ray showed almost complete opacification of the left hemithorax with a shift of the heart to the left, hemivertebrae of T4 to T7, scoliosis (Figure 1), and absence of the left radius. The baby's karyotype was 46XY. An electrocardiogram showed right axis deviation, right atrial enlargement, and right ventricular hyperFrom

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“…Isolation of the left subclavian artery occurs significantly more often than isolation of the RSA. 6 Most cases of isolation of the subclavian artery are associated with other congenital heart defects, especially when 22q11 microdeletion is present. 2,6 The 22q11 deletion is found in 81% of patients with subclavian artery isolation and is combined with an interruption of the aortic arch.…”

Section: Commentmentioning

confidence: 99%

Isolated Right Subclavian Artery With Interrupted Aortic Arch, Ventricular Septal Defect, and Left Ventricular Outflow Tract Obstruction

Hoven

Clur

J³

et al. 2015

World J Pediatr Congenit Heart Surg

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We present two cases of isolated right subclavian artery from the right pulmonary artery (PA) associated with interrupted aortic arch, ventricular septal defect, left ventricular outflow tract obstruction, and 22q11 microdeletion. Both patients were successfully managed with bilateral PA banding initially followed by a modified Yasui operation. Isolation of the subclavian artery is rare but should always be taken into account, especially when bilateral PA banding is considered. The banding must then be placed on the PA distal to the origin of the subclavian artery.

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Rare forms of isolation of the subclavian artery: echocardiographic diagnosis and surgical considerations

Cited by 28 publications

References 17 publications

Genetic heterogeneity and phenotypic anomalies in children with atrioventricular canal defect and tetralogy of Fallot

Genetic heterogeneity and phenotypic anomalies in children with atrioventricular canal defect and tetralogy of Fallot

Subclavian and Pulmonary Steal Phenomenon in Isolated Left Subclavian Artery with Left Lung Agenesis.

Isolated Right Subclavian Artery With Interrupted Aortic Arch, Ventricular Septal Defect, and Left Ventricular Outflow Tract Obstruction

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