Atrioventricular Block in Situs Ambiguus and Left Isomerism (Polysplenia Syndrome)

Roguin, Nathan; Pelled, B; Freundlich, E; Yahalom, Malka; Riss, E

doi:10.1111/j.1540-8159.1984.tb04853.x

Cited by 14 publications

(9 citation statements)

References 6 publications

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“…The excluded 22 reports were either case studies or case series. [15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33] We outline our methodology in Figure 1. The chosen reports included a total of 121 patients, of whom 52 had right isomerism and 69 left isomerism.…”

Section: Resultsmentioning

confidence: 99%

Chronic Arrhythmias in the Setting of Heterotaxy: Differences between Right and Left Isomerism

Loomba

Willes

Kovach

et al. 2015

Congenital Heart Disease

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So-called heterotaxy affects lateralization of the thoracic and abdominal organs. Congenital malformations may be present in one of several organ systems. Cardiac involvement includes both structural and conduction abnormalities. Data regarding arrhythmias in heterotaxy come from case reports and small case series. We pooled available data to further characterize arrhythmias in heterotaxy. A systematic review of the literature for manuscripts describing arrhythmias in heterotaxy patients was conducted. Databases including PubMed, EMBASE, and Ovid were searched. Studies describing arrhythmias in patients with heterotaxy were included if they were in English and presented characteristics of the arrhythmias. Arrhythmia characteristics were abstracted and are presented as pooled data. Freedom from arrhythmia by age was then analyzed using Kaplan-Meier analysis. A total of 19 studies with 121 patients were included in the pooled analysis. Those with right isomerism were found to be more likely to have atrial flutter, atrial tachycardia, junctional tachycardia, and ventricular tachycardia. Those with left isomerism were more likely to have atrioventricular block, intraventricular conduction delay, sick sinus syndrome, and atrioventricular nodal reentry tachycardia. Median age of onset for all arrhythmias was 4 years with no difference by specific arrhythmia or isomerism. Those with right and left isomerism are at risk for different arrhythmias but are likely to develop arrhythmias at the same age. Those with left isomerism are more likely to require pacemaker placement due to atrioventricular block. Understanding these differences allows for focused surveillance of development of these arrhythmias.

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Section: Resultsmentioning

confidence: 99%

Chronic Arrhythmias in the Setting of Heterotaxy: Differences between Right and Left Isomerism

Loomba

Willes

Kovach

et al. 2015

Congenital Heart Disease

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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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Atrioventricular Block in Situs Ambiguus and Left Isomerism (Polysplenia Syndrome)

Cited by 14 publications

References 6 publications

Chronic Arrhythmias in the Setting of Heterotaxy: Differences between Right and Left Isomerism

Chronic Arrhythmias in the Setting of Heterotaxy: Differences between Right and Left Isomerism

Disfunção sinusal em paciente com isomerismo atrial esquerdo

Special Imaging Casebook

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