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10. A Cyanotic Newborn with a Pink Right Upper Extremity

Author

Beichl, Matthias, Hanslik, Andreas, Zimpfer, Daniel, Rittenschober-Boehm, Judith, Nagl, Katrin, and Michel-Behnke, Ina

Subjects
Article Subject
Abstract

Aberrant origin of the subclavian artery (SCA) is a well-known vascular anomaly as part of congenital heart diseases with the left subclavian artery (LSCA) being more frequently affected than the right subclavian artery (RSCA). Complete isolation of the SCA is an even more infrequent aortic arch anomaly, occurring in less than 1% for the LSCA and even less for the RSCA. Isolation of the RSCA in patients with d-transposition of the great arteries (D-TGA) is even scanter with only a hand full of cases being reported in the literature. However, isolation of the RSCA has important implications on hemodynamics and surgical strategies. In this case report, we present a newborn patient with D-TGA which presented with distinct differential cyanosis. While the right upper extremity appeared pink with an oxygen saturation of 100%, the rest of the body was cyanotic. At first, this appearance was interpreted as the Harlequin phenomenon during primary care. However, detailed echocardiography revealed an aberrant origin of the RSCA from the right pulmonary artery, which led to the differential cyanosis. The patient underwent arterial switch operation on day of life two including dissection and reimplantation of the RSCA. The special hemodynamic situation of this is discussed in terms of pathophysiology and as well as its impact on perioperative and surgical management.

Published
2020
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11. Predictors of spontaneous closure of isolated secundum atrial septal defect in children: a longitudinal study

Author

Hanslik, Andreas, Pospisil, Ulrich, Salzer-Muhar, Ulrike, Greber-Platzer, Susanne, and Male, Christoph

Subjects
Atrial septal defects -- Diagnosis, Atrial septal defects -- Care and treatment, Children -- Health aspects
Abstract

OBJECTIVES. The goals were to assess the frequency of spontaneous closure of isolated secundum atrial septal defect in children and to identify predictors of spontaneous atrial septal defect closure. METHODS. A retrospective cohort study was performed in a tertiary care pediatric cardiology center. Consecutive patients (n = 200) diagnosed as having isolated atrial septal defects (no multiple or fenestrated atrial septal defects, no additional congenital heart disease, and no syndromes) were monitored for >6 months with serial 2-dimensional echocardiography, according to a standardized protocol. RESULTS. The median age at diagnosis was 5 months (minimum: 0 months; maximum: 13.9 years). The atrial septal defect diameter at diagnosis was 4 to 5 mm in 40% of cases, 6 to 7 mm in 28% of cases, 8 to 10 mm in 21% of cases, and >10 mm in 11% of cases. The median age at the final follow-up evaluation was 4.5 years (range: 6.8 months to 16.2 years). Thirty-four percent of atrial septal defects showed spontaneous closure, and 28% decreased to a diameter of [less than or equal to] 3 mm. Logistic regression analysis revealed atrial septal defect diameter and age at diagnosis as independent predictors of spontaneous closure or regression to [less than or equal to] 3-mm defect size. Of atrial septal defects with a diameter of 4 to 5 mm at diagnosis, 56% showed spontaneous closure, 30% regressed to a diameter of [less than or equal to] 3 mm, and none required surgical closure. Of atrial septal defects with a diameter of >10 mm at diagnosis, none closed spontaneously, whereas 77% required surgical or device closure. Gender and observation time were not associated with spontaneous atrial septal defect closure or regression to [less than or equal to] 3 mm. CONCLUSIONS. In the present study population of children with atrial septal defects, 62% showed spontaneous closure (34%) or regression to [less than or equal to] 3 mm (28%). Initial atrial septal defect diameter was the main predictor of spontaneous closure. Key Words atrial septal defect, congenital heart disease, spontaneous closure, natural course Abbreviations ASD--atrial septal defect BSA--body surface area OR--odds ratio CI--confidence interval, ATRIAL SEPTAL DEFECT (ASD) is among the most common types of congenital heart disease, with ostium secundum ASD representing the majority of cases. (1,2) Surgical closure is regarded as the [...]

Published
2006

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