A case report of late-onset symptoms of erythrocytosis in univentricular dextrocardia.

RATIONALE: Univentricular dextrocardia is a rare congenital heart disease that usually presents cyanotic manifestations from childhood. Due to the sustained dysfunction of blood oxygenation, it is very difficult to keep an asymptomatic survival. Herein, we described an interesting case of univentricular dextrocardia who suffered from initial symptoms in his middle age. PATIENT CONCERNS: A 54-year-old male patient with numbness and tingling of limbs was admitted to hospital due to the secondary manifestations of congenital heart disease. DIAGNOSIS: The patient was diagnosed as univentricular dextrocardia with pulmonary hypertension and secondary erythrocytosis based on computed tomography (CT) scan, echocardiography, and laboratory examinations. INTERVENTIONS: Intravenous hydration therapy with normal saline successfully eliminated his hyperviscosity associated symptoms. In view of socio-economic reasons, this patient refused surgical evaluation and further medical interventions. OUTCOMES: During 18-month follow up, he received no drug except for regular water intake. Fortunately, his life quality was satisfactory, and no other symptoms emerged except for mild numbness of limbs. LESSONS: In univentricular dextrocardia, it is possible to keep a long-term asymptomatic period due to the slow progress of pathophysiology. In this population, regular cardiac function evaluation and avoiding dehydration may help improve the quality of life.

Catheter ablation of supraventricular tachycardia in patients with dextrocardia and situs inversus.

BACKGROUND: Dextrocardia with situs inversus is a rare cardiac positional anomaly. Catheter ablation procedures performed in this set of patients have not been sufficiently reported. METHODS: A total of 10 patients with dextrocardia and situs inversus who received catheter ablation for supraventricular tachycardia (SVT) were included from a cohort of over 20 000 cases of catheter ablation for SVT in three centers from 2005 to 2016. All patients underwent electrophysiologic study and catheter ablation of SVT. Ablation targets were selected based on different tachycardia mechanisms with the primary endpoint of noninduction of tachycardia. RESULTS: The average age was 32.4 ± 5.6 years. Congenitally corrected transposition of great arteries (TGA) with situs inversus and D-looping of the ventricles and aorta (congenitally corrected TGA {I,D,D}) was found in four patients, while the other six patients exhibited mirror-image dextrocardia {I,L,L}. The mechanisms of SVT were atrioventricular nodal reentrant tachycardia in four patients, atrioventricular reentrant tachycardia in three, typical atrial flutter in one, intra-atrial reentrant tachycardia in one, and focal atrial tachycardia in one. Immediate procedural success was achieved in 9 out of 10 patients with no procedural complications. During a follow-up period of 6.3 ± 3.5 years on average, all patients remained free from recurrent tachycardia. CONCLUSIONS: For patients with dextrocardia and situs inversus, catheter ablation of SVT is safe and feasible. Differences in catheter maneuver and fluroscopy projection, along with difficulties in distorted anatomy are major obstacles for successful ablation.

A retrospective review of right-sided hearts at a South African tertiary hospital.

BACKGROUND: A right-sided heart (RSH) has three main causes: dextrocardia, dextroposition and dextroversion. It may be associated with cardiac malformation, extra-cardiac abnormalities and adverse patient outcomes. The aim of this study was to determine the prevalence, demographics, associated cardiac malformations (ACM) and outcome of paediatric patients diagnosed with a RSH at a South African tertiary hospital. METHODS: A retrospective review was performed over a 22-year period. RESULTS: RSH comprised 1% of the paediatric cardiology referrals. Dextrocardia was the most frequent cause (58.1%) and the majority of these patients had ACM (81.5%). More than a third (40.9%) were diagnosed with dextroposition, secondary to extra-cardiac factors. Dextroversion was the least common cause (1.1%). Over a quarter of all patients were confirmed dead at the time of the study; most had been diagnosed with dextrocardia. Two-thirds of the patients were lost to follow up. CONCLUSIONS: A RSH is an unusual occurrence. Dextrocardia, the most common cause, is frequently associated with ACM and extra-cardiac abnormalities. It is therefore important to timeously elucidate the cause of a RSH.

Partition of Common Atrioventricular Valve in a Patient With Dextrocardia and Univentricular Circulation.

Long-term outcomes in children with atrioventricular septal defect (AVSD) and univentricular palliation are of concern, with <60% survival at 25 years.1 Common atrioventricular valves (AVV) often become insufficient in patients with univentricular physiology, leading to heart failure.1,2 We have recently observed that outcomes of children with AVSD who reach Fontan circulation are not as bad as previously thought, provided that the AVV remains competent.1 Common AVV surgery is associated with substantial mortality and reoperation rates.3 Although successful AVV repair is associated with better survival and freedom from reoperation, good quality repair is difficult to achieve in univentricular circulation,3 especially in patients with dextrocardia.4 Herein, we report a patient with unbalanced AVSD and dextrocardia who underwent AVV repair using the "polytetrafluoroethylene (Gore-Tex, W.L. Gore & Associates, Flagstaff, AZ) bridge" technique5 with excellent early outcome.

Atrioventricular Canal and Dextrocardia: A Case Report with Implications for Community-Based Providers.

Congenital heart defects (CHD) are the most common congenital malformation reported in the literature, with a global incidence of eight per every 1,000 live births. In the United States approximately 40,000 infants are born each year with a CHD. Of the infants diagnosed with a CHD, one in every four heart defects are life threatening in origin. Early identification and treatment of congenital heart lesions, beginning with a comprehensive physical assessment after birth, are critical. For infants delivered at community-based hospitals, the importance of the physical assessment, timing of diagnostic strategies, anticipatory planning, and interprofessional collaboration among referring and accepting centers cannot be understated. This article presents a rare case of an infant with atrioventricular canal complicated by dextrocardia. Embryology, pathophysiology, epidemiology, symptomology, cardiac assessment, diagnostics, treatment, and nursing strategies for facilitating transfer of care from community-based hospitals to tertiary medical centers are discussed.

Quantitation in Dextrocardia on myocardial perfusion imaging: how to perform quantitative analysis using Cedars-Sinai software.

Dextrocardia, although a rare cardiac abnormality, carries the same risk for cardiac events as other people. SPECT Myocardial perfusion imaging is a potentially helpful diagnostic tool in patients with dextrocardia. Because of swapping of lateral and septal walls on SPECT slices, although visual analysis is possible, quantitation is substantially limited. Here, we introduce a simple practical method to make quantitative analysis feasible and accurate.

Severe aortic stenosis in dextrocardia with situs invertus and anomalous single coronary ostium treated with transcatheter aortic valve replacement.

Dextrocardia with situs inversus presents a unique anatomy with right-sided vascular system that may be associated with a number of additional cardiac and vascular malformations. A rare association is the presence of a single coronary artery ostium. To our knowledge, this is the first reported case of transcatheter aortic valve replacement using Edwards SAPIEN S3 valve in Dextrocardia patient with single coronary artery take off.

Clipping through a mirror: First reported successful MitraClip in a patient with dextrocardia.

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Successful percutaneous coronary intervention for an in-stent chronic total occlusion in a patient with dextrocardia: a case report.

BACKGROUND: Percutaneous coronary interventions of chronic total occlusion represent one of the most challenging issues in interventional cardiology. A Caucasian patient with dextrocardia presented with an in-stent chronic total occluded right coronary artery, a constellation which has not been described previously in the literature. CASE PRESENTATION: A 69-year-old man with pre-known situs inversus totalis and a long history of coronary artery disease with multiple interventions and stent-implantations presented to our department suffering from episodes of chest pain under exercise. A coronary angiogram showed a completely occluded right coronary artery in the area of a drug-eluting stent which had been implanted eight years before. We found collaterals from the left coronary artery system and signs of calcification, so the lesion was classified as chronic total occlusion and the recanalization using an antegrade wire escalation technique successfully performed. CONCLUSION: This is the first reported case of an in-stent chronic total occlusion in situs inversus with dextrocardia. Our experience in this case demonstrates the feasibility of recanalization of in-stent chronic total occlusions even in the rare setting of this congenital cardiac malposition combined with severe coronary artery disease.

Comparing levocardia and dextrocardia in fetuses with heterotaxy syndrome: prenatal features, clinical significance and outcomes.

BACKGROUND: To investigate the differences in cardiovascular disease, extracardiac anomalies and outcomes between fetuses with levocardia and dextrocardia. METHODS: Clinical demographics, prenatal features, postnatal characteristics and the outcomes of fetuses with levocardia or dextrocardia were recorded and analyzed. RESULTS: Sixty-five fetuses with dextrocardia and thirty-eight fetuses with levocardia were enrolled. Right ventricle outlet obstruction, atrioventricular septal defect and intestinal malrotation were common in both groups. Univentricular physiology, transposition of the great arteries and esophageal atresia were more frequent in fetuses with levocardia, whereas abnormal pulmonary venous connection, double outlet of right ventricle, left ventricle outlet obstruction and brain abnormalities were more frequent in the dextrocardia group. The accuracy of evaluating cardiac malformations was high, but the sensitivity in assessing extracardiac abnormalities was low. CONCLUSIONS: Although the disorders have certain overlapping features, there are several differences between fetuses with levocardia and dextrocardia. These findings might improve patient counseling and perinatal management.

Catheter ablation of premature ventricular contractions originating from aortic sinus cusps in a patient with dextrocardia and situs solitus: A case report.

BACKGROUND: Premature ventricular contractions (PVCs) originating from aortic sinus cusps is not infrequent and can be eliminated effectively by radiofrequency ablation with rare complications. However, after a review of the medical literature, and to our knowledge, this is the first case of successful idiopathic aortic sinus cusps-PVC-ablation using a 3-dimensional (3D) mapping system in an adult with dextrocardia. METHODS: A 62-year-old male with dextrocardia and situs inversus underwent catheter ablation of frequent PVCs. The electrocardiograms (ECG) were recorded by placement of the electrodes in reversed positions. The PVCs exhibited left bundle branch block and inferior axis QRS morphology with transition at leads V2-V3. The activation mapping indicated the earliest site of ventricular activation between the left and right aortic sinus cusps, highlighting that catheter ablation was successful at this point. RESULTS: The catheter ablation was successful between the left and right aortic sinus cusps, and the PVCs were not detected for the subsequent 30 min following the procedure as well as for the rest of the hospital stay. CONCLUSION: Combined with ECG electrodes in reversed positions and 3D electroanatomical mapping system, catheter ablation of PVCs originating from aortic sinus cusps in patients with dextrocardia can be safely and effectively performed.