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At least 37 records · Page 2Linked to original sources

Poland-Möbius syndrome associated with dextrocardia.

A newborn male with Möbius syndrome, Poland anomaly, and dextrocardia is described. This is the second case reported of Poland-Möbius syndrome associated with dextrocardia. The patient presented with strabismus, facial diplegia, difficulty in swallowing, hypoplasia of the left pectoralis major muscle, partial absence of the upper costal cartilages, absence of the left areola, hypoplasia of the left forearm and hand, and dextrocardia without murmurs.

Abnormalities, Multiple↗

[Myocardial revascularization in dextrocardia with situs inversus].

BACKGROUND: The incidence of coronary artery disease in patients with dextrocardia associated with situs inversus is similar to that of the general population: Nevertheless, there are few papers regarding surgical myocardial revascularization in these patients. METHODS: We report two patients with dextrocardia and situs inversus who underwent myocardial revascularization by means of coronary artery bypass grafting, with the right internal mammary artery to the left anterior descending coronary artery in one case and with the left internal mammary artery as a free graft to the left anterior descending coronary artery associated with the repair of an aneurysm of the right coronary sinus of Valsalva in the second patient. RESULTS: There were no surgical complications; the patients were discharged on the 4th and 7th postoperative days, respectively. They are alive and well after 24 and 60 months of follow-up respectively. CONCLUSIONS: This report suggests that durable myocardial revascularization in dextrocardia with situs inversus can be successfully achieved with right or left mammary artery bypass grafting to the left anterior descending coronary artery. The advantage of using an in situ mammary artery is discussed.

Aged↗

[Coronary artery bypass in dextrocardia with situs inversus totalis--a case report].

A 71-year-old woman was admitted to our hospital by ambulance, because of right chest pain and hypotension. Chest X-ray and standard 12-lead ECG showed mirror-image dextrocardia with situs inversus totalis. ECG with right-left reversal of all leads showed acute inferior myocardial infarction. The patient underwent coronary bypass surgery due to postinfarction angina, and she is now doing well 2 years following the operation. Mirror-image dextrocardia with situs inversus totalis is very unusual. But the patients are believed to have normal longevity, and presumably have an incidence of atherosclerotic coronary artery disease similar to the general population. To our knowledge, this is the first case of coronary bypass surgery on a patient with mirror-image dextrocardia in Japan.

Aged↗

[An experience of successful valve repair for acquired mitral and tricuspid regurgitation in dextrocardia, situs inversus, bilateral vena cava, and hemiazygos continuation].

A 56-year-old woman was admitted to our department because of congestive heart failure. Chest X-ray showed dextrocardia with situs inversus. The echocardiography revealed severe mitral regurgitation due to prolapse of posterior leaflet and secondary Venography demonstrated the bilateral superior vena cava, huge coronary sinus and hemiazygos continuation with hypoplastic inferior vena cava. Mitral valve regurgitation was repaired by Carpentier's and Burr's technique, and tricuspid valve regurgitation was repaired by Kay's technique. Her postoperative course was uneventful. Valve surgery for acquired valvular lesion in dextrocardia with situs inversus is rare. Although valve repair is easily performed even in mirror-image heart as well as in normal, levo-position heart, preoperative evaluation of systemic venous return is important in the case of dextrocardia with systemic venous anomalies.

Azygos Vein↗

Mitral valve replacement in a Jehovah's Witness with dextrocardia and situs solitus.

BACKGROUND AND AIMS OF THE STUDY: Dextrocardia with situs solitus and mitral valve insufficiency requiring surgical treatment is a rare presentation. Jehovah's Witnesses (JW), a specific religious group, deny any blood transfusion and for this reason the cardiac surgeon has to plan his operation well in advance, particularly in the case of adhesions from previous thoracic procedures. MATERIALS AND METHODS: A 50-year-old white female Jehovah's Witness with dextrocardia and situs solitus was referred for surgical treatment of massive mitral valve insufficiency of rheumatic etiology. Due to multiple adhesions from previous bilateral thoracotomies and the inverted position of the heart, cardiopulmonary bypass (CPB) was initiated with an aortic and a left common femoral vein cannulae. CPB was completed with an additional SVC cannula. The surgeon, having excellent exposure from the opposite side of the table, was able to perform a mitral valve replacement (MVR) with a 31 mm St. Jude Medical valve prosthesis, through a giant left atrium under moderate hypothermia and crystalloid cardioplegia. The operation was bloodless, with only two units of autotransfused blood being used with a postoperative hematocrit of 34%. RESULTS: The patient had an uneventful recovery and has been in NYHA class I for 24 months now. CONCLUSIONS: The case is presented for the safety of the approach, the excellent exposure from the left side in a dextrocardia case and the avoidance of blood transfusion in a Jehovah's patient.

Blood Transfusion, Autologous↗

Dextrocardia and Marfan's syndrome.

It is described the occurrence of dextrocardia together with the congenital cyanotic heart disease in 20 year old man included in the fruste forme of the Marfan's syndrome. The diagnosis was made by the physical examination with the evidence of the arachnodactyly by the metacarpal indices and confirmed by autopsy with the following results: dextrocardia, large atrial septal defect, common ventricle, atresia of the pulmonary artery with the collateral lung perfusion from the descending aorta. There were found neither ocular manifestations, nor unambiguous manifestations of the aortic lesions. The ultrastructural examinations showed only greater accumulation of the PAS positive substances. Dextrocardia as the cardiovascular manifestation of the Marfan's syndrome has not yet been reported in the available literature.

Adult↗

Specifics of technique in percutaneous mitral commissurotomy in a case of dextrocardia and situs inversus with mitral stenosis.

Percutaneous mitral commissurotomy (PMC) was successfully performed in a 48-year-old woman with dextrocardia and situs inversus and a severe mitral stenosis. After PMC, the valve area increased from 0.97 to 1.89 cm2, and no complication occurred. This case shows that PMC is practicable in a case of dextrocardia with situs inversus. The procedure appears to be safer and easier when carried out with the inoue balloon, image inversion on the screen, and under transesophageal echocardiographic monitoring.

Catheterization↗

Dextrocardia with and without situs viscerum inversus in two sibs.

We described two sibs born to consanguineous Sicilian parents who died of severe congenital heart malformation. Both had dextrocardia; however, only the girl had situs viscerum inversus. At necropsy she was found to have a right spleen and right pulmonary isomerism (three lobes in each lung, as commonly found in the asplenia syndrome). This observation, together with other literature reports, suggest that isolated dextrocardia, situs viscerum inversus, and the asplenia-polysplenia complex may be different end results of a unique dysmorphogenetic process involving the embryonic midline.

Abnormalities, Multiple↗

Percutaneous balloon valvuloplasty in mirror-image dextrocardia and rheumatic mitral stenosis.

A 28-year-old woman with mirror-image dextrocardia and severe rheumatic mitral stenosis underwent successful percutaneous balloon mitral valvuloplasty (PBMV). Standard technique was altered to include transseptal catheterization via the left femoral vein and inter-atrial septal puncture with the transseptal needle rotated to a 7 o'clock position. The predilatation transmitral gradient (mean) of 16 mmHg decreased to 5 mmHg following PBMV. The valve area improved from 1.0 cm2 to 2.6 cm2. There were no complications. This case illustrates that transseptal catheterization can be accomplished safely in patients with unusual cardiac anatomy and mitral valvuloplasty can be performed in patients with mirror-image dextrocardia and rheumatic mitral stenosis.

Adult↗

Double-inversion technique for coronary angiography viewing in dextrocardia.

This report describes a simple angiographic viewing rule for coronary angiography in patients of dextrocardia with obstructive coronary artery disease, which could correct the unfamiliar angulated pictures of the coronary tree in dextrocardia into the familiar conventional angiographic pictures of a normally located heart and its associated ease of interpretation.

Coronary Angiography↗

Dextrocardia and corrected transposition of the great arteries (I,D,D) in a case of Kartagener's syndrome: a unique association.

Kartagener's syndrome (KS) usually includes mirror-image dextrocardia. The incidence of congenital heart disease in KS is comparable with that in the general population. This paper reports on a case of Kartagener's syndrome associated with dextrocardia, corrected transposition of the great arteries (I,D,D), ventricular septal defect, and valvar pulmonary stenosis in an 8-year-old girl.

Cardiac Catheterization↗

Pulmonary agenesis associated with dextrocardia, sternal defects, and ectopic kidney.

Pulmonary agenesis is a rare embryological defect, usually unilateral, and is associated with skeletal, cardiovascular, and other anomalies. A 22-month-old girl was admitted to our clinic with recurrent pulmonary infections. A chest X-ray and multidetector computed tomography (MDCT) revealed pulmonary agenesis with dextrocardia, sternal defects, and a right pelvic ectopic kidney. We report on the first known case of right-sided pulmonary agenesis combined with isolated dextrocardia, sternal defects, and an ectopic kidney. The importance of MDCT for the diagnosis of pulmonary agenesis and associated other anomalies is demonstrated.

Dextrocardia↗

[Dextrocardia and Poland syndrome in a 59-year-old patient].

Poland syndrome consists of unilateral absence of the sternal head of the pectoralis major muscle, ipsilateral symbrachydaktylia, and occasionally associated other malformations of the anterior chest wall, mammilla, and mamma. To our knowledge a simultaneous occurrence with dextrocardia was reported in seven patients. We report on an additional patient with this unusual coincidence. A 59-year old man showed mild symbrachydaktylia of the left hand and deformity of the left part of the anterior chest wall, both present since birth. We found an additional dextrocardia with situs solitus, d-loop, and correctly connected great arteries. Besides arterial hypertension there was no remarkable impairment of the patient's condition.

Dextrocardia↗

Two-dimensional echocardiographic assessment of dextrocardia: a segmental approach.

Two-dimensional echocardiography was used in the prospective evaluation of 40 patients with the clinical diagnosis of dextrocardia. A segmental analysis of the situs, connections, ventricular anatomy, and chamber positions was utilized for a complete diagnostic assessment. An adequate examination was possible in 33 of these patients; the findings were confirmed by cardiac catheterization and angiography in 31 patients and at operation in 26. Use of the location of the liver and the drainage of the hepatic veins and inferior vena cava allowed atrial visceral situs to be defined in 33 patients (solitus 21, inversus 9, and ambiguous 3). Pulmonary venous connections were correctly identified in 27. In 33 patients, atrioventricular (AV) and ventriculoarterial connections and ventricular anatomy were correctly predicted. Twenty patients had 2 separate well-developed ventricles. Ventriculoarterial connections were determined correctly in all 20 patients: concordant in 5, discordant in 6, double-outlet right ventricle in 5, and single-outlet right ventricle (pulmonary atresia) in 4. In 16 patients a ventricular septal defect was correctly identified. In the remainder the ventricular septum was intact. Thirteen patients had univentricular heart: 8 had 2 AV valves (double-inlet ventricle) 3 had common AV inlet, and 2 had atresia of 1 AV connection. Two-dimensional echocardiography allowed the accurate assessment of complex congenital heart defects associated with dextrocardia. Utilizing a segmental approach, one can correctly predict atrial-visceral situs, ventricular morphology and situs, and AV and ventriculoarterial connections.

Adolescent↗

Placement of a left ventricular assist device in a patient with dextrocardia.

Dextrocardia most commonly presents in the setting of situs inversus, but it may occur as an isolated anomaly with normal position of the abdominal organs. Herein we present a 54-year-old man with ischemic cardiomyopathy and dextrocardia with normal position of the abdominal organs who presented with an exacerbation of congestive heart failure requiring inotropic support as well as mechanical ventilation. An implantable, wearable left ventricular assist device was placed in this patient to allow for ambulation and eventual discharge home. The patient survived 4 months before he developed pneumonia and expired.

Comorbidity↗

Radiofrequency catheter ablation of a right free-wall accessory pathway in dextrocardia with complete situs inversus.

An uncommon case of orthodromic atrioventricular reentrant tachycardia in a patient with dextrocardia and complete situs inversus is reported. There was no associated cardiac abnormality apart from the mirror image dextrocardia. An electrophysiological study and successful catheter ablation of a right free-wall concealed accessory pathway was performed. A simplified three-catheter technique from a femoral approach and monoplane fluoroscopy were used in a noncomplicated procedure of normal duration.

Catheter Ablation↗

A case of dextrocardia, radial ray malformation and renal anomaly.

A 12-year-old boy is described with bilateral radial club hands, scoliosis, hypospadias, isolated dextrocardia, hypoplastic ribs, an ectopic kidney and spina bifida occulta. Although some of the clinical features of this patient are seen in VATER association and sacrococcygeal dysgenesis, the presence of dextrocardia, facial dysmorphism, radial, renal and vertebral anomalies preclude these diagnoses.

Abnormalities, Multiple↗

Radiofrequency catheter ablation of a coronary sinus-ventricular accessory connection in dextrocardia with complete situs inversus and an anomalous inferior vena cava.

An anomalous inferior vena cava (IVC) is often associated with patients with dextrocardia. However, radiofrequency catheter ablation in such a case with that combination has not been reported. We encountered a case of Wolff-Parkinson-White syndrome with dextrocardia associated with complete situs inversus and an azygos continuation of the IVC. A steep angulation at the junction of the azygos vein and superior vena cava precluded the precise mapping of the anatomical right cavity with a femoral vein approach. Successful catheter ablation of an accessory connection between the coronary sinus and left ventricle could be achieved with a right cubital vein approach.

Catheter Ablation↗