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British Heart Journal Nov 1987A twenty year old man is described who has a left-sided liver, a single right-sided spleen, an anomalous inferior vena cava with azygos continuation, and a...
A twenty year old man is described who has a left-sided liver, a single right-sided spleen, an anomalous inferior vena cava with azygos continuation, and a morphologically normal left-sided heart. These findings emphasise the pitfalls in predicting cardiac abnormalities from other aberrations of anatomical situs.
Topics: Adult; Heart Defects, Congenital; Humans; Levocardia; Liver; Male; Spleen; Vena Cava, Inferior
PubMed: 3676045
DOI: 10.1136/hrt.58.5.534 -
Journal of the American College of... Jul 1984A 2 year old boy with ventricular inversion and normal ventriculoarterial connection is described. Associated cardiac lesions included single atrium, absence of the...
A 2 year old boy with ventricular inversion and normal ventriculoarterial connection is described. Associated cardiac lesions included single atrium, absence of the coronary sinus, bilateral superior venae cavae, absence of the hepatic segment of the inferior vena cava with azygos and hemiazygos continuation, right aortic arch, levocardia and left atrial isomerism. At 5 days of age, the patient underwent a Waterston (aortopulmonary) anastomosis because of suspected pulmonary atresia. The correct diagnosis was established at 2 years of age and the patient had a successful Mustard operation (interatrial baffle procedure) and closure of the Waterston anastomosis. Accurate preoperative diagnosis is difficult in this rare cardiac anomaly and the reported mortality rate is high.
Topics: Cardiac Catheterization; Child, Preschool; Echocardiography; Electrocardiography; Heart Atria; Heart Defects, Congenital; Heart Ventricles; Humans; Male; Methods; Radiography
PubMed: 6736440
DOI: 10.1016/s0735-1097(84)80331-9 -
British Heart Journal Apr 1981Sixty-two hearts without a patent exit from the left atrium to the ventricular mass have been studied. All had situs solitus and laevocardia. The material consisted of...
Sixty-two hearts without a patent exit from the left atrium to the ventricular mass have been studied. All had situs solitus and laevocardia. The material consisted of 32 cases with coexistent aortic atresia and 30 cases with patent aortic root. Five hearts with aortic atresia were biventricular with atrioventricular concordance and imperforate left atrioventricular valves, and 27 hearts were univentricular of right ventricular type, with absent left atrioventricular connection. The anatomy of this group was uniform, with extreme hypoplasia of all the left cardiac segments. Among the cases with patent aortic root, five were biventricular, with atrioventricular concordance and imperforate left atrioventricular valves, and 24 had absent left atrioventricular connection, 15 with univentricular heart of right ventricular type and nine with univentricular heart of left ventricular type. The final heart had double inlet univentricular of left ventricular type with an imperforate left atrioventricular valve. In this second group the aorta was larger in cases with discordant ventriculoarterial connection or in those with double outlet from the main ventricular chamber. A normal sized aorta without aortic arch obstruction was observed in nine instances. These are of great interest in terms of surgical anatomy since definitive palliation may be feasible.
Topics: Aorta; Aortic Valve; Female; Heart Atria; Heart Ventricles; Humans; Infant; Infant, Newborn; Male; Mitral Valve; Prognosis
PubMed: 7225254
DOI: 10.1136/hrt.45.4.393 -
British Heart Journal Mar 1981We have analysed the atrioventricular junction, ventricular mass, and ventriculoarterial junction in 43 hearts with isomeric atrial chambers. Of the hearts, 32 had...
We have analysed the atrioventricular junction, ventricular mass, and ventriculoarterial junction in 43 hearts with isomeric atrial chambers. Of the hearts, 32 had atrial chambers of bilateral right morphology while 11 had atrial chambers with bilateral left atrial characteristics. Among the hearts with right atrial isomerism, there were 13 biventricular hearts, all with ambiguous atrioventricular connection. Eight had a common valve and five had two atrioventricular valves. In the other 19 hearts, the atrial chambers were connected to only one ventricular chamber, 18 having double inlet ventricle through a common valve and the other having absence of the left atrioventricular connection. In these univentricular hearts, all possible types of ventricular morphology were found. The ventriculoarterial junction among these hearts with right isomerism showed great variation. In the hearts with left atrial isomerism, nine hearts had two ventricles and two were univentricular. The biventricular hearts all had ambiguous atrioventricular connection, six via a common valve and three via two valves. The two univentricular hearts both had double inlet via a common valve, one to a chamber of right ventricular type and the other to a chamber of left ventricular type. Both had rudimentary chambers of complementary pattern. The ventriculoarterial junction again showed much variation. Statistical analysis showed that pulmonary obstruction and a univentricular heart were both significantly more frequent in association with right compared with left isomerism. Significant differences were also noted in the two groups in terms of ventriculoarterial connections and infundibular morphology.
Topics: Aorta; Dextrocardia; Heart Atria; Heart Valves; Heart Ventricles; Humans; Levocardia; Myocardium; Pulmonary Artery
PubMed: 7470338
DOI: 10.1136/hrt.45.3.236 -
Archives of Disease in Childhood Oct 1978In a case of laevocardia, bronchiectasis, and paranasal sinus abnormalities, assessment on 2 occasions showed the presence of moderate immunodeficiency. Serum...
In a case of laevocardia, bronchiectasis, and paranasal sinus abnormalities, assessment on 2 occasions showed the presence of moderate immunodeficiency. Serum concentrations of IgG and IgM were low, and serum and salivary IgA was not detected. T-lymphocytes were reduced in number and cell-mediated immunity in vivo and in vitro was impaired. Opsonisation, complement system, and neutrophil functions were normal.
Topics: Bronchiectasis; Child; Female; Heart Defects, Congenital; Humans; Immunologic Deficiency Syndromes; Levocardia; Paranasal Sinuses
PubMed: 727796
DOI: 10.1136/adc.53.10.814 -
British Heart Journal Aug 1976Aortic valve atresia is usually associated with severe hypoplasia of the left ventricle and mitral apparatus. Palliative procedures, including atrial septectomy,...
Aortic valve atresia is usually associated with severe hypoplasia of the left ventricle and mitral apparatus. Palliative procedures, including atrial septectomy, pulmonary artery-aorta anastomosis, and pulmonary artery banding, have been performed in some patients. However, because of the distinct underdevelopment of the left heart, the long-term prognosis must be guarded even in the survivors of these procedures. Rarely, aortic valve atresia is found with adequate left heart chambers. Because very few of these patients are described, the clinical, angiocardiographic, and the pathological findings in two patients are presented, with comments on palliation in the neonatal period, and the potential for ventriculoaortic reconstitution.
Topics: Aortic Valve; Cineangiography; Echocardiography; Electrocardiography; Female; Heart Defects, Congenital; Humans; Infant, Newborn; Levocardia; Male; Palliative Care; Prognosis
PubMed: 61758
DOI: 10.1136/hrt.38.8.821 -
British Heart Journal Jul 1966
Topics: Adolescent; Adult; Angiocardiography; Child; Child, Preschool; Dextrocardia; Electrocardiography; Female; Follow-Up Studies; Heart Septal Defects; Humans; Male; Situs Inversus; Spleen
PubMed: 5942468
DOI: 10.1136/hrt.28.4.472 -
British Heart Journal Jan 1965
Topics: Angiocardiography; Child; Dextrocardia; Heart Defects, Congenital; Humans; Infant; Levocardia; Prognosis
PubMed: 14242166
DOI: 10.1136/hrt.27.1.69 -
British Heart Journal Nov 1963
Review
Topics: Birth Weight; Consanguinity; Dextrocardia; Embryology; Genetics, Medical; Heart Defects, Congenital; Humans; Levocardia; Seasons; Situs Inversus
PubMed: 14072604
DOI: 10.1136/hrt.25.6.803 -
British Heart Journal Jun 1960
Topics: Dextrocardia; Genetic Diseases, X-Linked; Heart Defects, Congenital; Heterotaxy Syndrome; Humans; Levocardia; Medical Records; Situs Inversus
PubMed: 13855788
DOI: 10.1136/hrt.22.3.429