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Successful surgical repair of solitus, dextrocardia, atrioventricular discordance, and double outlet right ventricle with l-malposition of the aorta.

The authors report a rare case of situs solitus of viscera and atria, dextrocardia, atrioventricular discordance, atrial and ventricular septal defects, origin of both great arteries from the morphologic right ventricle, pulmonary valvar and subvalvar stenosis and l-malposition of the aorta which has been correctly diagnosed and succesfully operated on. The repair was accomplished by closing the ventricular septal defect through a ventriculotomy in the systemic venous ventricle (morphologic left), and placing a conduit between this ventricle and the pulmonary artery which had been ligated proximally. The conduit was a fresh segment of homograft valved ascending aorta fixed on a Dacron woven tube. The authors discuss: firstly the reasons for performing this type of repair instead of the intraventricular one recommended by the Mayo Clinic group in the past and secondly the peculiar location of the conducting tissue and the method of avoiding it when approaching the ventricular septal defect.

Aorta↗

Dextrocardia and ventricular septal defect in the Möbius syndrome.

A case of Möbius syndrome is reported with the additional features of dextrocardia and a single ventricle, neither of which have been previously reported in the literature with this syndrome. Correlation of the period in embryogenesis of cardiac partitioning and rotation with the appearance of the cranial nuclei and limb buds would support the proposal that the fourth to the seventh week of gestation is the critical period of embryogenesis, wherein an intrauterine insult could give rise to the Möbius syndrome.

Abnormalities, Multiple↗

Successful repair of double-outlet right ventricle, complete atrioventricular canal, and atrioventricular discordance associated with dextrocardia and pulmonary stenosis.

The association of complete atrioventricular canal with other complex congenital cardiac anomalies has represented a significant challenge for the cardiac surgion. The combination of double-outlet right ventricle with complete atrioventricular canal has been particularly difficult to correct, with no surgical successes having been reported until recently. This is a report of the first successful repair of double-outlet right ventricle, complete atrioventricular canal, and atrioventricular discordance (ventricular incersion) associated with common atrium, bilateral superior venae cavae, dextrocardia, and pulmonary stenosis. The specialized conduction tissue was identified by intracardiac electrophysiological mapping, and normal sinus rhythm was preserved. Postoperative cardiac catheterization showed excellent hemodynamics. One year postoperatively, the patient was attending school, playing swimming without difficulty, and taking no cardiac medications. This good result lends encouragement for considering total repair for similar patients with the combination of double-outlet right ventricle, complete atrioventricular canal, and other associated congenital cardiac anomalies.

Angiocardiography↗

Acrocephalosyndactyly with hydrocephalus and dextrocardia in a Nigerian child.

A rare case of dextrocardia and hydrocephalus in a patient with acrocephalosyndactyly is reported. In the patient presented here, barium meal studies showed the gastrointestinal tract to be in normal position. There was no evidence of cardiac disease but the patient showed some neurological retardation at age nine months.

Abnormalities, Multiple↗

[Atherosclerotic ischemic cardiopathy in patients with dextrocardia in situs viscerum inversus].

We describe the incidence, clinical, radiologic, electrocardiographic, echocardiographic and angiographic findings of two cases with dextrocardia in situ viscerum inversus with ischaemic heart disease. The first patient had coronary artery saphenous bypass graft and is currently asymptomatic with a negative maximal stress test. The other patient with diabetes mellitus and unfavorable coronary anatomy was not operated and is currently with stable angina on class II of the NYHA. The electrocardiographic, echocardiographic and angiographic strategies are commented.

Cardiac Catheterization↗

Dextrocardia and situs inversus in Sri Lanka.

Transposition of the viscera is an uncommon condition but no figures are available as to its prevalence in a tropical country. 31 cases of dextrocardia were seen among 110,603 persons examined at two chest clinics in Sri Lanka. A further 11 cases were detected by the reading of films taken at a tuberculosis prevalence survey in the general population in which 27,095 persons were examined after random selection. The incidence at the chest clinics was 1 in 3567, while the prevalence in the general population was 1 in 2500, perhaps the highest rate to be reported from any country. The prevalence was higher in the rural than in the urban population. All cases were right handed, implying that there was no transposition of the centres in the brain. In one case the mother had mumps in the first trimester of pregnancy, thus raising the possibility of an aetiological relationship.

Dextrocardia↗

[Emergent coronary artery bypass grafting in a patient with mirror-image dextrocardia].

A 49 year-old-man with known situs inversus totalis was admitted to our hospital with a history of angina pectoris of 4 months duration. Coronary arteriography revealed that the left main trunk had a 90% stenosis. After the arteriography, the patient's hemodynamics was deteriorated, and he required a support of intraaortic balloon pumping. He underwent emergent coronary artery bypass grafting with saphenous veins the following day, and recovered uneventfully. Technical problems in this patient from a surgical viewpoint were discussed. As far as we know, this is the first case of coronary bypass grafting on a patient with mirror-image dextrocardia and a stenosis of the left main trunk.

Coronary Artery Bypass↗

[Simultaneous coronary artery bypass grafting and total arch graft replacement with dextrocardia and situs inversus].

The patient was a 79-year-old male who complained a sudden back pain. He had dextrocardia and situs inversus. An aortogram showed the dilatation of the ascending and aortic arch which reached 5.6 cm in diameter. A coronary angiogram also showed the 75 percent stenosis of segment 3 in the right coronary artery. Coronary artery bypass grafting to the right coronary artery was performed using a saphenous vein graft during cooling stage of the extracorporeal circulation. After that total arch replacement was carried out using a graft with four side branches with an aid of selective cerebral perfusion. He complicated with respiratory failure postoperatively and needed tracheostomy but recovered gradually. A postoperative computed tomogram showed good patency of three side grafts for cerebral perfusion and no abnormality of graft anastomosis.

Aged↗

Straddling mitral valve with hypoplastic right ventricle, crisscross atrioventricular relations, double outlet right ventricle and dextrocardia: morphologic, diagnostic and surgical considerations.

The clinical, surgical and morphologic findings in five cases of a rare form of straddling mitral valve are presented. Three patients were diagnosed by two-dimensional echocardiography, cardiac catheterization and angiocardiography and two had diagnostic confirmation at autopsy. All five cases shared a distinctive and consistent combination of anomalies: 1) dextrocardia; 2) visceroatrial situs solitus, concordant ventricular D-loop and double outlet right ventricle with the aorta positioned to the left of and anterior to the pulmonary artery; 3) hypoplasia of right ventricular inflow (sinus) with tricuspid valve stenosis or hypoplasia; 4) large right ventricular infundibulum (outflow); 5) malalignment conoventricular septal defect; 6) straddling mitral valve with chordal attachments to the left ventricle and right ventricular infundibulum; 7) severe subpulmonary stenosis with well developed pulmonary arteries; and 8) superoinferior ventricles with crisscross atrioventricular (AV) relations. The degree of malalignment between the atrial and ventricular septa was studied quantitatively by measuring the AV septal angle projected on the frontal plane. The AV septal angle in the two postmortem cases was 150 degrees, reflecting marked malalignment of the ventricles relative to the atria. This AV malalignment appears to play an important role in the morphogenesis of straddling mitral valve. As judged by a companion study of seven postmortem cases, the more common form of straddling mitral valve with a hypertrophied and enlarged right ventricular sinus had less severe ventricular malposition than did the five rare study cases with hypoplastic right ventricular sinus. A competent mitral valve, low pulmonary vascular resistance and low left ventricular end-diastolic pressure were found at cardiac catheterization in the three living patients who underwent a modified Fontan procedure and are doing well 2.2 to 5.8 years postoperatively.

Adult↗

Off-pump coronary artery bypass grafting in a high-risk dextrocardia patient: a case report.

We present an interesting but high-risk case of an obese male patient aged 56 years with dextrocardia and a left diaphragmatic hernia. Anterior myocardial infarction was diagnosed in 1994, and the patient later presented with a history of unstable angina. The diagnosis for this chronic smoker was triple-vessel disease, impaired left ventricular function, chronic renal failure, chronic bronchitis, impaired lung function, pulmonary hypertension, hypertension, diabetes, and chronic active gastritis (EuroSCORE of 10). The patient underwent successful off-pump coronary artery bypass grafting with 3 saphenous vein grafts to the left anterior descending, obtuse marginal, and right posterior descending arteries. He was discharged home 8 days later.

Coronary Artery Bypass, Off-Pump↗

Total correction of double-outlet right ventricle, atrial septal defect, pulmonic stenosis with a concordant atrioventricular relationship, dextrocardia and situs inversus.

Surgical correction of a rare type of double-outlet right ventricle was successfully carried out in a 20-year-old man. The malformation was associated with atrial septal defect, ventricular septal defect, pulmonic stenosis, a concordant atrioventricular relationship, and dextrocardia with situs inversus. A Teflon patch was sutured as a baffle in the right ventricle to direct blood that had passed from the left ventricle through the ventricular septal defect out through the aorta. The infundibular obstruction was resected, a pulmonic commissurotomy performed and the atrial septal defect closed. The patient made an uneventful recovery and was well at follow-up 1 year later.

Journal Article↗