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.

Dextrocardia in Adults with Congenital Heart Disease.

BACKGROUND/OBJECTIVES: Dextrocardia is rare in the general population, and may be associated with significant additional cardiac malformations. We aimed to identify the prevalence and patterns of additional cardiac defects, as well as the associated long-term morbidity and mortality, in adult patients with dextrocardia, in a specialised Adult Congenital Heart Disease (ACHD) service. METHODS: A retrospective study of patients with dextrocardia managed by our tertiary ACHD service, since January 2000, was performed. Medical records were reviewed and the National Death Index was consulted to confirm survival status. RESULTS: Of 3698 adults in our ACHD Service, 19 (0.5%) had dextrocardia. Mean follow-up duration was 7±7.5 years. The mean age at last review was 36.8±10.5 years (range 20-63 years). Situs was solitus in 14 (74%) and inversus in five (26%). Eleven patients (58%) had functional single ventricles, of whom five had atrioventricular (AV)-ventriculoarterial (VA) discordance and two had VA discordance only. Four patients with two ventricles had AV-VA discordance. All patients had at least one additional cardiac malformation. Fourteen patients (74%) required surgical intervention. Eleven patients (58%) underwent a Fontan-type operation. Five patients (26%) required ablation procedures for arrhythmia. One patient had infective endocarditis and two deaths occurred, both in patients who also had AV-VA discordance. CONCLUSION: Dextrocardia remains a rare finding in adults, even in a highly select group of patients with known congenital heart disease. Those with associated congenital heart abnormalities are likely to have complex lesions, which may require multiple surgical and medical interventions. Despite this, our series demonstrated that patients surviving to adulthood and then managed in an ACHD centre may have good medium-term survival.

Surgical palliation in patients with a single ventricle and dextrocardia.

BACKGROUND: Dextrocardia is found in a significant proportion of patients undergoing a single-ventricle repair. Surgical outcomes in this cohort are unclear. METHODS: The records of 41 consecutive patients with single-ventricle physiology and dextrocardia presenting in a single institution from 1990 to 2008 were reviewed. Of this cohort, 19 patients had heterotaxy syndrome. Twenty-five of the 41 patients had atrioventricular valve regurgitation (AVVR) on presentation (mild, 13; moderate, 9; severe, 3). RESULTS: One patient died before intervention. Initial surgical palliation was performed in 31 patients. Four patients died postoperatively and 4 interim deaths occurred between initial palliation and bidirectional cavopulmonary shunt (BCPS). Thirty of the surviving 32 patients underwent BCPS, with 2 perioperative deaths. There were 4 additional deaths before Fontan surgery. Twenty-two of the surviving 25 patients underwent a Fontan procedure. There was 1 postoperative mortality. Survival to the age of 15 years was 56% (95% confidence interval [CI], 39%-70%). Patients with moderate or severe regurgitation had higher mortality if they were managed conservatively rather than by surgery (5 of 6 vs 2 of 6; P=.24). Patients with bilateral BCPS had better operative outcomes and survival compared with peers with unilateral anastomosis (odds ratio, 27; P=.005; 95% CI, 2.7-269). The side of the systemic venous pathway did not seem to influence outcomes. CONCLUSIONS: Surgical outcomes of single-ventricle palliation seem poor in patients with dextrocardia. Aggressive management of congenital AVVR might improve the long-term prognosis.

Experience with bidirectional cavopulmonary anastomosis and modified Fontan operation in patients with single ventricle and concomitant visceral heterotaxy.

The object of our study was to analyze the results of bidirectional cavopulmonary anastomosis (BCPA) and modified Fontan operations (MFO) in patients with a functionally single ventricle and heterotaxy syndrome and to reveal risk factors for these surgical interventions. During 1983-2010, 681 patients underwent BCPA or MFO. Thirty-nine had heterotaxy syndrome. The median follow-up period after BCPA and MFO was nine and 1.5 years, respectively. Risk factors for lethal outcomes were determined by logistic regression analysis. Hospital mortality after BCPA and MFO was 7.9% and 12.5%, respectively and did not significantly differ from patients without heterotaxy. The most frequent hospital complications were heart failure, pleural effusions, and arrhythmias. Late mortality after BCPA and MFO was 8.7% and did not significantly differ from patients without heterotaxy. Late deaths were caused by congestive heart failure or pulmonary thromboembolism. The main non-lethal complication was arrhythmia. Patients have significantly improved their functional class at follow-up. The independent risk factor for lethal outcomes after BCPA and MFO was preoperative regurgitation at atrioventricular valves (P=0.012). BCPA and MFO in patients with a functionally single ventricle and heterotaxy syndrome allow to significantly improves their quality of life. Preoperative regurgitation at atrioventricular valves worsens surgical results.

Increased postoperative and respiratory complications in patients with congenital heart disease associated with heterotaxy.

OBJECTIVE: Patients with heterotaxy and complex congenital heart disease underwent cardiac surgery with high mortality and morbidity. Recent studies have revealed an association among heterotaxy, congenital heart disease, and primary ciliary dyskinesia. We undertook a retrospective review of patients undergoing cardiac surgery at Children's National Medical Center between 2004 and 2008 to explore the hypothesis that there is increased mortality and respiratory complications in heterotaxy patients. METHODS: Retrospective review was performed on postsurgical outcomes of 87 patients with heterotaxy and congenital heart disease exhibiting the full spectrum of situs abnormalities associated with heterotaxy. As controls patients, 634 cardiac surgical patients with congenital heart disease, but without laterality defects, were selected, and surgical complexities were similar with a median Risk Adjustment in Congenital Heart Surgery-1 score of 3.0 for both groups. RESULTS: We found the mean length of postoperative hospital stay (17 vs 11 days) and mechanical ventilation (11 vs 4 days) were significantly increased in the heterotaxy patients. Also elevated were rates of tracheostomies (6.9% vs 1.6%; odds ratio, 4.6), extracorporeal membrane oxygenation support (12.6% vs 4.9%: odds ratio, 2.8), prolonged ventilatory courses (23% vs 12.3%; odds ratio, 2.1) and postsurgical deaths (16.1% vs 4.7%; odds ratio, 3.9). CONCLUSIONS: Our findings show heterotaxy patients had more postsurgical events with increased postsurgical mortality and risk for respiratory complications as compared to control patients with similar Risk Adjustment in Congenital Heart Surgery-1 surgical complexity scores. We speculate that increased respiratory complications maybe due to ciliary dysfunction. Further studies are needed to explore the basis for the increased surgical risks for heterotaxy patients undergoing cardiac surgery.

Heterotaxy syndrome: defining contemporary disease trends.

The purpose of this study was to define a population of visceral heterotaxy and to investigate the incidence of bacterial sepsis in the current era of universal pediatric pneumococcal immunization. Pediatric echocardiography and radiology databases, along with electronic medical records, were searched for patients followed-up since birth between 1999 and 2009 with either asplenia or polysplenia and cardiac anatomy consistent with heterotaxy syndrome. A total of 29 patients were identified. Seven patients (24%) had a total of 8 sepsis events, and 6 patients (86%) developed sepsis while taking appropriately prescribed antibiotic prophylaxis. Of the patients with sepsis, 5 had polysplenia and 2 had asplenia. Sixty-two percent of sepsis events were nosocomially acquired. No cases of pneumococcal sepsis occurred after the introduction of the conjugated pneumococcal vaccination to the pediatric vaccination schedule. Bacterial sepsis was associated with a 44% mortality rate. An unexpected finding in 3 patients with visceral heterotaxy, asplenia, and an interrupted inferior vena cava (IVC) as the only anomaly on echocardiography was associated intestinal malrotation. Children with visceral heterotaxy remain at significant risk of life-threatening bacterial infection. In addition, the finding of interrupted IVC on echocardiography should prompt screening for intestinal malrotation, even in the absence of additional structural heart disease.

Dextrocardia and situs inversus totalis in a Nigerian cadaver: a case report of rare anomaly / Dextrocardia y situs inversus totalis en un cadáver nigeriano: reporte de caso de una rara anomalía

This report describes an adult male cadaver with dextrocardia and situs inversus totalis in a Nigerian cadaver. The photographic illustrations revealed transposition of some of the visceral organs such that the stomach and spleen were located on the right and the liver and gall bladder on the left. Also, the left lung was tri-lobed while the right was uni-lobed. The heart was flattened and flipped to the right thus, transposing the great vessels. The report showed that dextrocardia and situs inversus exists amongst Nigerians and possibly Africans and therefore wish to recommend early medical examination since patients with this condition are unaware of their unusual anatomy until they seek medical attention for an unrelated condition. Early detection may lead to a successful surgical management and consequently offer a safer chance of survival.

Este reporte describe a un cadáver adulto nigeriano de sexo masculino con dextrocardia y situs inversus totalis. Las ilustraciones fotográficas revelaron transposición de algunas de las vísceras, como el estómago y el bazo que se encuentra en el derecho y el hígado y la vesícula biliar a la izquierda. Además, el pulmón izquierdo era tri-lobulado, mientras que el derecho uni-lobulado. El corazón fue aplanado y situado a la derecha, por consiguiente, transposicionando los grandes vasos. El reporte demostró que la dextrocardia y el situs inversus existen entre los nigerianos y, posiblemente, los africanos, por tanto, se recomienda un temprano examen médico, ya que pacientes con esta condición no son conscientes de su inusual anatomía hasta que acuden al médico por atención por una condición no relacionada. La detección temprana puede conducir a un éxito quirúrgico y, en consecuencia, ofrecer una segura oportunidad de sobrevivir.

A population-based study of cardiac malformations and outcomes associated with dextrocardia.

The incidence of dextrocardia and its associated cardiac and noncardiac malformations is not known. There is inadequate information about outcomes to counsel parents about prognosis. A retrospective review of all diagnoses of dextrocardia due to embryologic development at a tertiary care hospital from 1985 to 2001 was performed. Eighty-one cases were identified (48 antenatally). The incidence of dextrocardia was estimated to be 1 in 12,019 pregnancies. Twenty-seven cases were situs solitus, 30 situs inversus, and 24 situs ambiguous or isomerism. Cardiac malformations were found in 26 of 27 cases of situs solitus, 7 of 30 cases of situs inversus, and 24 of 24 cases of isomerism. Noncardiac malformations were identified in 10 of 27 cases of situs solitus, 6 of 30 cases of situs inversus, and 14 of 24 cases of isomerism. Twelve pregnancies were terminated, 3 fetuses were stillborn, and 2 women chose compassionate care. All terminated fetuses were diagnosed with dextrocardia before termination, and all had >1 cardiac anomaly; 7 also had noncardiac anomalies. There were 43 subjects in the intention-to-treat group (20 situs solitus, 10 solitus inversus, 13 isomerism). Thirty-two had >or=1 cardiac operation, and 21 had >or=3. Thirty-nine subjects were alive at most recent follow-up. In conclusion, the incidence of dextrocardia was 1 in 12,019 pregnancies. In conclusion, in our cohort, the numbers of cases of situs solitus, situs inversus, and isomerism were similar. Cardiac and noncardiac malformations were most common in the isomerism group. Cardiac malformations were often complex in the situs solitus and isomerism groups. Ninety-one percent of those in the intention-to-treat cohort were alive at follow-up.