Left isomerism and complete atrioventricular block: A report of six cases

García, Otto L.; Mehta, Ashok V.; Pickoff, Arthur S.; Tamer, Dolores; Ferrer, Pedro L.; Wolff, Grace S.; Gelband, Henry

doi:10.1016/0002-9149(81)90327-1

Cited by 53 publications

(19 citation statements)

References 10 publications

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“…A-V block with left isomerism (polysplenia syndrome) who had ECD and complex congenita l heart disease (Garcia et al, 1981). However, in adu lts, the development of nonsurgical complete A-V block in ECD appears to have been recognized more frequently.…”

Section: F'g I Sequential Standa Rd Electrocardiographic Lead Ri Rhmentioning

confidence: 99%

Acquired nonsurgical complete atrioventricular block in a child with endocardial cushion defect

Mehta

O'Riordan

Sánchez

et al. 1982

Clinical Cardiology

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Summary: Acquired nonsurgical complete atrioventricular block is uncommon in children. We report an ll-year-old asymptomatic girl with endocardial cushion defect who gradually developed progressive conduction abnormality and complete atrioventricular block prior to open heart surgery. To our knowledge, a gradual progressive, nonsurgical, acquired atrioventricular block in endocardial cushion defect has not been described before. This report discusses the possible pathogenesis of acquired nonsurgical complete atrioventricular block in endocardial cushion defect and reviews the literature.Key words: first degree A-V block, Mobitz type I 2° A-V block, Mobitz type II 2° A-V block

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Section: F'g I Sequential Standa Rd Electrocardiographic Lead Ri Rhmentioning

confidence: 99%

Acquired nonsurgical complete atrioventricular block in a child with endocardial cushion defect

Mehta

O'Riordan

Sánchez

et al. 1982

Clinical Cardiology

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“…Three types of heart rhythm disturbances may occur in the isomerism of the left atrial appendages: sinus node dysfunction 5 , atrioventricular block [6][7][8] , and dual atrioventricular nodal pathways 5 .…”

Section: Discussionmentioning

confidence: 99%

“…At fifteen years of age, 70% of the patients present with sinus bradycardia. Atrioventricular block may be present in 50% of the patients in some phase of their lives, and may be progressive [6][7][8] , with a higher prevalence of atrioventricular block in the fetus 9 .…”

Section: Polysplenia Was First Described By Abernethy In 1793mentioning

confidence: 99%

Disfunção sinusal em paciente com isomerismo atrial esquerdo

Sobrinho¹,

Moscardini²,

Croti³

et al. 2006

Arq. Bras. Cardiol.

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We report the case of an asymptomatic six-year-old child with left atrial isomerism and sinus venosus atrial septal defect. The physical examination revealed several periods of bradycardia. During a 24-hour electrocardiographic monitoring the patient presented a significant sinus node dysfunction with sinus pauses of up to 2.4 seconds. A permanent pacemaker was implanted, with a satisfactory outcome.Mailing address: Sírio hassem Sobrinho • Rua Siqueira Campos, 2597/13 -15025-055 • São José do Rio

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“…1 Approximately 20% of patients with left-isomerism syndrome have congenital AV block. [1][2][3] Approximately 13 patients have been described in the English literature with left-isomerism syndrome and congenital AV block. Several other patients with acquired AV block and other complex bradycardias have also been described.…”

Section: Denouement and Discussion Left-isomerism (Polysplenia) With mentioning

confidence: 99%

“…Several other patients with acquired AV block and other complex bradycardias have also been described. 2,3 Abnormalities of cardiac conduction are common in patients with left-isomerism syndrome. In these patients, the sinoatrial node is usually hypoplastic, ectopic, or absent.…”

Section: Denouement and Discussion Left-isomerism (Polysplenia) With mentioning

confidence: 99%

Special Imaging Casebook

Herman

Siegel²

1999

J Perinatol

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A 3200-gm infant girl was born at term to a 22-year-old mother. The infant was noted to have marked bradycardia after the delivery and a heart rate of 45 beats per minute. An electrocardiogram examination performed at the outside hospital demonstrated complete heart block. A chest radiograph at that institution demonstrated dextrocardia. The patient was transferred to St. Louis Children's Hospital for placement of an epicardial dual-chamber pacemaker. Cardiac sonogram at the time of admission confirmed dextrocardia and demonstrated an atrial septal defect, ventriculoseptal defect, and patent ductus arteriosus, and a pacemaker was placed ( Figure 1). Because of persistent conjugated hyperbilirubinemia, the child was evaluated by abdominal sonogram ( DENOUEMENT AND DISCUSSION Left-Isomerism (Polysplenia) with Congenital Atrioventricular Block and Biliary AtresiaThe patient had many malformations associated with left-isomerism (polysplenia) syndrome, including two with significant morbidity: congenital complete atrioventricular (AV) block and biliary atresia. In addition to these abnormalities, left-isomerism syndrome is associated with bilateral bilobed lungs, bilateral left-sided hilar bronchioarterial relationships (pulmonary arteries passing superior to the main stem bronchus referred to as hyparterial bronchi), transverse liver with a horizontal inferior margin, intestinal malrotation, multiple spleens, and intrahepatic interruption of the inferior vena cava with azygos (or occasionally hemiazygos continuation to the superior vena cava). Cardiac lesions are common, including those of the bilateral superior vena cava. Endocardial cushion defect and partial anomalous pulmonary venous return are also common. 1 However, unlike right-isomerism (asplenia) syndrome, the great vessels are usually normally related and pulmonic stenosis is uncommon. 1 Approximately 20% of patients with left-isomerism syndrome have congenital AV block. 1-3 Approximately 13 patients have been described in the English literature with left-isomerism syndrome and congenital AV block. Several other patients with acquired AV block and other complex bradycardias have also been described. 2,3 Abnormalities of cardiac conduction are common in patients with left-isomerism syndrome. In these patients, the sinoatrial node is usually hypoplastic, ectopic, or absent. The coronary sinus node, which develops from the left sinus horn, 1 often takes over. The P-wave in patients with leftisomerism syndrome is abnormally superior and leftward in the frontal plane. This unusually directed P-wave may help differentiate between left-and right-isomerism syndromes.

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Left isomerism and complete atrioventricular block: A report of six cases

Cited by 53 publications

References 10 publications

Acquired nonsurgical complete atrioventricular block in a child with endocardial cushion defect

Acquired nonsurgical complete atrioventricular block in a child with endocardial cushion defect

Disfunção sinusal em paciente com isomerismo atrial esquerdo

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