Case 4/2017 - Double-Chambered Right Ventricle with Dextrocardia and Hypoxemia Due to Atrial Shunt in a 4-Year-Old Girl

Double-chambered Right Ventricle in Children

“…16 Of the remaining 9 patients, all had one or more systolic murmurs. The heart murmur was audible at the left sternal boarder in 4 (44.4%) patients, 9,13,14,17 at the right sternal boarder in 3 (33.3%) patients, 10,22,24 and at precordium 20 and at all foci 12 in 1 (11.1%) patient, respectively. Among 6 patients with an electrocardiogram examined, 2 (33.3%) patients had sinus tarchycardia, 9,13 1 (16.7%) patient had normal sinus rhythm, 22 and 3 (50%) patients showed signs of left/right ventricular hypertrophy or left atrial enlargement.…”

“…In total, 18 articles reporting pediatric DCRV were collected, 4,8-24 with 3 original articles, 4,8,15 1 case series; 18 and 14 case reports included. [9][10][11][12][13][14]16,17,[19][20][21][22][23][24] As a result, as many as 62 pediatric patients were recruited in this study. One of the patients was a fetus, who was diagnosed with DCRV and also delivered at 28-week gestation, and died at the age of 6 hours.…”

“…Among 6 patients with an electrocardiogram examined, 2 (33.3%) patients had sinus tarchycardia, 9,13 1 (16.7%) patient had normal sinus rhythm, 22 and 3 (50%) patients showed signs of left/right ventricular hypertrophy or left atrial enlargement. 10,12,14 The locations/natures of the hypertrophied anomalous muscle bands were heterogeneously described for 30 patients (Table II).…”

“…An intra-right ventricle pressure gradient was measured by echocardiography in 51 (51/62, 82.3%) patients, 4,[8][9][10][11][12][13][14][15][16][17]20,23,24 and by catheterisation in 27 (27/62, 43.5%) patients, 4,8,15 with a mean pressure gradient of 92.8 ±27.6 (rang, 45-141; median, 86) mmHg (n=17).…”

“…Between the thin-walled infundibulum and the inflow 1 (3.4) segment of the right ventricle 16 abnormal in 4 (66.7%) patients ( 2 =1.3, p=0.567) [including a hypertrophied anomalous muscle at the infundibular region (n=2), 11,12 a narrow infundibulum due to hypertrophy (n=2), 10 and a thin-walled infundibulum (n=1). 16 Ventricular hypertrophy was seen in 12 (12/62, 19.4%) patients: it was right ventricular hypertrophy in 10 (83.3%) patients, biventricular hypertrophy in 1 (8.3%) patient, and biventricular hypertrophy with right atrial enlargement in 1 (8.3%) patient, respectively ( 2 =20.3, p<0.001).…”