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Dextrocardia with situs solitus and severe mitral regurgitation is a rare clinical presentation which posse a surgical challenge and requires specific preoperative planning. A 54-year-old women with this anatomy, multiple thoracic procedures, and severe mitral valve regurgitation underwent successful mitral valve replacement with a 27-mm mechanical prosthesis through a left thoracotomy under ventricular fibrillation, on the basis of computed tomography findings. We emphasize the importance of preoperative planning and a surgical approach through a left thoracotomy and under ventricular fibrillation.

We report a case of a 12-yr-old boy referred to our unit with congenital generalized lipodystrophy and dilated cardiomyopathy related to a lamin gene mutation. He progressively developed end-stage heart failure and was referred for heart transplant evaluation. The patient's lipid profile, glucose level, and renal function were normal, and vascular retinopathy was ruled out. He underwent orthotopic bicaval HT and had an uneventful recovery. He was discharged home two wk after surgery with good graft function. During follow-up, he developed hyperglycemia and dyslipidemia, which were controlled by increasing leptin dose and starting oral antidiabetic drugs. The patient is currently doing well two yr after transplantation.

Objective(s) To assess the impact of univentricular versus biventricular pacing (BiVP) on systemic ventricular function in patients with congenitally corrected transposition of the great arteries (ccTGA). Methods We performed a retrospective review of all patients with a diagnosis of ccTGA who underwent pacemaker insertion. From 1993 to 2014, 53 patients were identified from the cardiology database and surgical records. Results Overall mortality was 7.5% (n = 4). One patient required transplantation and 3 late deaths occurred secondary to end-stage heart failure. Median follow-up was 3.7 years (range, 4 days to 22.5 years). Twenty-five (47%) underwent univentricular pacing only, of these, 8 (32%) developed significant systemic ventricular dysfunction. Twenty-eight (53%) received BiVP, 17 (26%) were upgraded from a dual-chamber system, 11 (21%) received primary BiVP. Fourteen (82%) of the 17 undergoing secondary BiVP demonstrated systemic ventricular dysfunction at the time of pacer upgrade, with 7 (50%) demonstrating improved systemic ventricular function after pacemaker upgrade. Overall, 42 (79%) patients underwent univentricular pacing, with 22 (52%) developing significant systemic ventricular dysfunction. In contrast, the 11 (21%) who received primary BiVP had preserved systemic ventricular function at latest follow-up. Conclusions Late-onset systemic ventricular dysfunction is a major complication associated with the use of univentricular pacing in patients with ccTGA. All patients with ccTGA who develop heart block should undergo primary biventricular pacing, as this prevents late systemic ventricular dysfunction. Preemptive placement of BiVP leads at the time of anatomical repair or other permanent palliative procedure will facilitate subsequent BiVP should heart block develop.

Objective Early results for anatomic repair of congenitally corrected transposition of the great arteries (ccTGA) are excellent. However, the development of left ventricular dysfunction late after repair remains a concern. In this study we sought to determine factors leading to late left ventricular dysfunction and the impact of cardiac resynchronization as a primary and secondary (upgrade) mode of pacing. Methods From 1992 to 2012, 106 patients (median age at surgery, 1.2 years; range, 2 months to 43 years) with ccTGA had anatomic repair. A retrospective review of preoperative variables, surgical procedures, and postoperative outcomes was performed. Results In-hospital deaths occurred in 5.7% (n = 6), and there were 3 postdischarge deaths during a mean follow-up period of 5.2 years (range, 7 days to 18.2 years). Twelve patients (12%) developed moderate or severe left ventricular dysfunction. Thirty-eight patients (38%) were being paced at latest follow-up evaluation. Seventeen patients had resynchronization therapy, 9 as an upgrade from a prior dual-chamber system (8.5%) and 8 as a primary pacemaker (7.5%). Factors associated with left ventricular dysfunction were age at repair older than 10 years, weight greater than 20 kg, pacemaker implantation, and severe neo-aortic regurgitation. Eight of 9 patients undergoing secondary cardiac resynchronization therapy (upgrade) improved left ventricular function. None of the 8 patients undergoing primary resynchronization developed left ventricular dysfunction. Conclusions Late left ventricular dysfunction after anatomic repair of ccTGA is not uncommon, occurring most often in older patients and in those requiring pacing. Early anatomic repair and cardiac resynchronization therapy in patients requiring a pacemaker could preclude the development of left ventricular dysfunction.

Patients who have undergone the Fontan procedure in later adolescence and adulthood represent a unique population at risk for significant morbidity and mortality. The optimal strategy for long-term management of such patients is unknown. The aim of this study was to evaluate outcomes of patients who had undergone Fontan surgery later in life, focusing on late survivorship, mode of death, and predictors of mortality. Eighty-eight patients were identified who had their initial Fontan operation from 1973 to 2007 at ≥15 years of age. A standardized tiered contact protocol was followed to capture the recent health status of each patient; the probability of survival was 83%, 71%, and 66% at 5, 10, and 15 years of follow-up, respectively. Despite focused efforts, the modes of death were not available in 48% of the patients. A prolonged intensive care unit stay at the time of operation was the single predictor of mortality (p = 0.0123). In conclusion, this investigation highlights the significant mortality that exists in patients who undergo a Fontan procedure later in life and the difficulties in achieving standardized medical follow-up for this high-risk group of patients.

Background— The outcome of the surgical repair in congenital heart disease correlates with the degree of myocardial damage. In this study, we determined whether mitochondrial DNA depletion is a sensitive marker of right ventricular (RV) damage and whether impaired mitochondrial DNA (mtDNA) replication contributes to the transition from compensated hypertrophy to failure. Methods and Results— RV samples obtained from 31 patients undergoing cardiac surgery were compared with 5 RV samples from nonfailing hearts (control). Patients were divided into compensated hypertrophy and failure groups, based on preoperative echocardiography, catheterization, and/or MRI data. Mitochondrial enzyme activities (citrate synthase and succinate dehydrogenase) were maintained during hypertrophy and decreased by ≈40% ( P P Conclusions— Impaired mtDNA replication causes early and progressive depletion of mtDNA in the RV of the patients with congenital heart disease during the transition from hypertrophy to failure. Decreased mtDNA content probably is a sensitive marker of mitochondrial injury in this patient population.

OBJECTIVE: To estimate the degree of association between domestic violence -physical, verbal or sexual- with suicidal behavior among university students. MATERIAL AND METHODS: A matched case-control study was done with students attending the University of Colima, Mexico. The cases were 235 teenagers who presented both suicidal ideation and suicide attempt; the controls were 470 individuals of the same age and sex. RESULTS: Sexual abuse showed the highest degree of association with suicidal behavior (OR= 27.4), followed by verbal violence (OR= 9.28), drug use (OR= 8.6), physical violence (OR= 5.5) and smoking (OR= 3.6). Multivariate logistic regression showed that verbal violence was associated with suicidal behavior independently of the other variables, while physical violence, sexual abuse, smoking and drug use seem to depend on verbal violence. CONCLUSIONS: Domestic violence, particularly verbal or sexual, is strongly associated with suicidal behavior in adolescents and should be considered in suicide prevention programs.

Background The efficacy of extracorporeal membrane oxygenation (ECMO) in bridging children with unrepaired heart defects to a definitive or palliative surgical procedure has been rarely reported. The goal of this study is to report our institutional experience with ECMO used to provide preoperative stabilization after acute cardiac or respiratory failure in patients with congenital heart disease before cardiac surgery. Methods A retrospective review of the ECMO database at Children's Hospital Boston was undertaken. Children with unrepaired congenital heart disease supported with ECMO for acute cardiac or respiratory failure as bridge to a definitive or palliative cardiac surgical procedure were identified. Data collection included patient demographics, indication for ECMO, details regarding ECMO course and complications, and survival to hospital discharge. Results Twenty-six patients (18 male, 8 female) with congenital heart disease were bridged to surgical palliation or anatomic repair with ECMO. Median age and weight at ECMO cannulation were, respectively, 0.12 months (range, 0 to 193) and 4 kg (range, 1.8 to 67 kg). Sixteen patients (62%) survived to hospital discharge. Variables associated with mortality included inability to decannulate from ECMO after surgery (p = 0.02) and longer total duration of ECMO (p = 0.02). No difference in outcomes was found between patients with single and biventricular anatomy. Conclusions Extracorporeal membrane oxygenation, used as a bridge to surgery, represents a useful modality to rescue patients with failing circulation and unrepaired complex heart defects.

The aim of this study was to determine the effect of thiamine pyrophosphate (TPP) on serum lactate levels, maximum oxygen consumption (Vo2max) and heart rate in male athletes performing aerobic activity. A double-blind, randomized, crossover study was performed in which lactate levels, Vo2max and heart rates in 27 male athletes were compared at rest and after exercise, following administration of placebo (sodium chloride 0.9%) or TPP (1 mg/kg). At rest, serum lactate levels after placebo or TPP were similar; however, after exercise, the levels were lower in the athletes after taking TPP than after placebo. During exercise, Vo2max in athletes on TPP was higher than on placebo. At rest, heart rate after taking placebo or TPP was similar but, after exercise, heart rate was lower after taking TPP than after placebo. It is concluded that TPP caused serum lactate levels and heart rate to be lower than placebo and Vo2max to be higher in athletes performing aerobic physical activity.

Objectives The goal of our study was to report our intermediate-term results with aortic root translocation plus arterial switch for d-transposition of the great arteries with left ventricular outflow tract obstruction. Background A d-transposition of the great arteries with left ventricular outflow tract obstruction represents a difficult surgical problem. The Rastelli procedure is the usual approach to this condition. However, recurrent left ventricular outflow tract obstruction and early conduit obstruction as well as arrhythmias and troublesome late mortality are significant limitations. Methods From 1993 to 2005, 11 children (8 male, 3 female) ages 1 month to 11 years (median age 7 months) have undergone aortic root autograft translocation plus arterial switch to correct d-transposition of the great arteries with left ventricular outflow tract obstruction. The native aortic root was excised from the right ventricle infundibulum and inserted into the left ventricular outflow, enlarging the outflow tract by resecting the outlet septum and an appropriate-size ventricular septal defect patch. After coronary artery reimplantation, right ventricular outflow reconstruction was achieved with a homograft. Results There were no early or late deaths. With a median follow-up of 59 months (range 2 to 137 months), 5 patients required 6 conduit replacement procedures at a median time of 53 months. Two patients required an implantable defibrillator for ventricular arrhythmias. None of the patients have developed left ventricular outflow tract obstruction. Conclusions Aortic root autograft plus arterial switch procedure is a good option for the surgical management of infants and children with d-transposition of the great arteries and left ventricular outflow tract obstruction and results in a more anatomic repair compared with Rastelli operation. Intermediate-term results indicate good relief of left ventricular outflow tract obstruction and need for conduit replacement compares favorably with the Rastelli procedure for this lesion.

Background Early results for anatomic repair of congenitally corrected transposition of the great arteries are excellent with respect to right ventricular and tricuspid valve function. However, development of left ventricular (systemic ventricle) dysfunction late after repair remains a concern. In this study we sought to determine factors leading to late impairment in left ventricular performance. Methods From August 1992 to July 2005, 44 patients (median age at surgery, 1.6 years; range, 0.6 to 39.6 years) with congenitally corrected transposition of the great arteries had anatomic repair. Left ventricular function and mitral regurgitation were evaluated by echocardiography at follow-up. Twenty-three patients had a Rastelli procedure, and 21 underwent an arterial switch. Twelve patients (27%) were pacemaker dependent at latest follow-up. Results Early mortality was 4.5% (n = 2) with 1 late death as a result of leukemia. Median follow-up was 3.0 years (range, 7 days to 12.4 years). Left ventricular function remained unchanged (normal) in 35 patients, improved in 1 patient, and deteriorated in 8 patients (18%). Mitral regurgitation was unchanged in 30 patients, improved in 6 patients, and worsened in 8 patients (18%). Development of left ventricular dysfunction was significantly associated with pacemaker implantation ( p = 0.005) and a widened QRS (>20% > 98% percentile of normal; p = 0.03). Conclusions Anatomic repair of congenitally corrected transposition can be performed with low operative mortality. However, late left ventricular dysfunction is not uncommon, with higher incidence in those requiring pacing and with a prolonged QRS. Resynchronization may be of value in patients requiring a pacemaker.

A 69-year-old man with no history of chest trauma was admitted to our institution complaining of blunt and severe central chest pain of cataclysmic onset radiating to the back. His medical history was remarkable only for a chronic atrial fibrillation treated with warfarin and amiodarone. On arrival, his ECG showed atrial fibrillation at 120 bpm with no signs of myocardial ischemia. Laboratory examination was uneventful except for an international normalized ratio of 7. With the presumptive diagnosis of acute aortic syndrome, a thoracoabdominal high-resolution contrast-enhanced computed tomography scan was performed that showed a large mass at the level of the left atrium (LA) and a moderate pericardial effusion (Figure 1 and Movie I in the online-only Data Supplement). Linear attenuation coefficients of the LA mass and pericardial fluid were suggestive of acute clot and blood (60 and 50 Hounsfield units, respectively). No signs of malignancy, pulmonary embolism, or aortic disease were found. During evaluation, our patient's clinical condition …

Aortico-left ventricular tunnel (ALVT) is a rare congenital paravalvar communication between the aorta and the left ventricle. This anomaly has been associated with the right coronary artery (RCA) arising directly from the tunnel. Surgical repair of this later condition is challenging and is usually undertaken by closure of the ventricular and aortic ends of the tunnel and transfer of the RCA. Regardless of the surgical technique, development of early and late aortic regurgitation (AR) remains a concern. We report a neonate with ALVT and the RCA coming off the tunnel that was successfully managed by closure of both ends of the tunnel, reinforcement of the annulus with autologous pericardium, and leaving the RCA perfused by way of the tunnel. After 6 months of follow-up, no AR has been shown on echocardiography.

Association of d-transposition of the great arteries and complete atrio-ventricular canal constitutes an uncommon and complex cardiac anomaly usually associated with poor prognosis. We report our experience on one-stage neonatal repair for d-transposition of the great arteries and complete atrio-ventricular canal. Between August 1997 and 2005, four patients (two males and two females) underwent anatomical correction for d-transposition of the great arteries and complete atrio-ventricular canal using an arterial switch procedure and two-patch repair. Mean age and weight at operation were 20 days (range from 3 to 28 days) and 3.2kg (range from 2.7 to 3.5kg), respectively. None of the patients received preoperative palliative procedure. Associated lesions were left outflow tract obstruction in three patients and multiple muscular ventricular septal defects in two patients. All four patients survived the operation. There was one in-hospitality death due to fungal sepsis. One patient required late re-operation for left ventricular outflow tract obstruction and left atrio-ventricular valve regurgitation. For a mean follow-up of 67 months (range from 51 to 90 months) all patients are asymptomatic and with no residual defects. Corrective repair of d-transposition of the great arteries and complete atrio-ventricular canal can be successfully achieved in this very challenging population during the neonatal period.

Q fever is characterized by its clinical polymorphism. Cardiac involvement in acute Q fever is rare. We report a case of pleuro-pericarditis that rapidly evolved to pericardial constriction during an acute episode of Coxiella burnetii infection. Constrictive pericarditis was confirmed by hemodynamic measurements, echocardiography, and magnetic resonance. Indirect immunofluorescence assay revealed positive serology for acute Q fever. The patient underwent a successful pericardiectomy and was given antibiotics. The histopathologic study of the excised pericardium showed C. burnetii in a large cluster of organisms. After a 6-month follow-up period, the individual was asymptomatic.

Combined aortic and pulmonic stenosis is a very rare condition, with only a few patients reported in the literature.1 Moreover, most of those cases are children and young adults, and thus, the pathogenesis has been often considered congenital.2,3 We report an 81-year-old man with a diagnosis of severe aortic and pulmonic stenosis who underwent successful aortic and pulmonic valve replacement, and we comment on the echocardiographic and pathological findings and speculate on the most likely cause. To the best of our knowledge, this is the first report of this condition in an octogenarian patient. An 81-year-old man with a medical history of smoking, arterial hypertension, dyslipidemia, mild renal …

The present study aimed to identify potential differences in hemodynamic performance between the supra-annular CarboMedics Top Hat valve and the intra-annular CarboMedics standard valve in terms of the long-term left ventricular mass reduction and transvalvular gradients.We retrospectively reviewed a series of 186 consecutive patients who had undergone aortic valve replacement with a small size mechanical prosthesis at our institution from 2003 to 2013, receiving either a CarboMedics Top Hat valve (53 patients, valve size, 21 mm in 52.8% and 23 mm in 47.2%) or a CarboMedics standard prosthesis (133 patients, valve size, 19 mm in 14.3% and 21 mm in 85.7%).The in-hospital mortality was 9.4% and 11.3% in the Top Hat and standard groups, respectively (P = .71). The mean percentage of left ventricular mass reduction was greater in the Top Hat group (33% ± 15.8% vs 20.1% ± 16.6%, P.001). The mean postoperative peak aortic gradient was lower in the Top Hat group (19.9 ± 8.9 vs 29.6 ± 8.6 mm Hg; P.001). Spearman analysis showed a positive correlation between the indexed effective orifice area and the percentage of left ventricular mass reduction (Rho = +0.65, P = .02). The survival in the Top Hat group was 79.7% and 71.7% at 5 and 10 years, respectively. In the standard group, survival was 66.8% and 61.5% at 5 and 10 years, respectively (log-rank test, 0.19). Cox regression demonstrated severe myocardial hypertrophy (hazard ratio, 2.559; 95% confidence interval, 1.095-5.981) as one of the independent predictors of survival.The Top Hat valve surpasses hemodynamically the intra-annular valve. We suggest the supra-annular Top Hat prosthesis can be especially recommended for patients with a small aortic root and severe myocardial hypertrophy.

ObjectivesNeonatal surgery for tetralogy of Fallot and truncus arteriosus requires reconstruction of the right ventricular outflow tract. Although the method of reconstruction is often dictated by the individual anatomy, choices exist. This review examines the durability and outcomes of right ventricular outflow tract reconstruction in the neonate.MethodsThis is a retrospective review of all 278 neonates with the diagnosis of tetralogy of Fallot and truncus arteriosus undergoing right ventricular outflow tract reconstruction at a single center between 1990 and 2007. Diagnostic variants included tetralogy of Fallot/pulmonary stenosis (n = 83), tetralogy of Fallot/pulmonary atresia (n = 81), and tetralogy of Fallot with absent pulmonary valve (n = 17). Truncus arteriosus was present in 97 patients. Patients were analyzed on the basis of diagnosis and the method of right ventricular outflow tract reconstruction: aortic homograft, pulmonary homograft, transannular patch, transannular patch with monocusp pulmonary valve, and nontransannular patch. Freedom from reoperation/reintervention was determined by using the log-rank test.ResultsThe mean age at right ventricular outflow tract reconstruction was 11.8 ± 8 days, and hospital survival was 95.0% for the tetralogy of Fallot group and 90.7% for the truncus arteriosus group. Overall freedom from reoperation and reintervention was 76.2% ± 14.8% in the nontransannular patch group and 59.5% ± 6.8% in the transannular patch group; both were significantly greater than seen in patients receiving either aortic (0%) or pulmonary (6.7% ± 4.2%) homografts (P < .05). There was no difference between aortic and pulmonary homografts. Among patients with tetralogy of Fallot/pulmonary stenosis, there was no difference in 10-year freedom from reoperation/reintervention between the transannular (70.8% ± 7.4%) and nontransannular patch methods (76.2% ± 14.8%, P = .53). At 10 years, the diagnosis of tetralogy of Fallot/pulmonary stenosis was associated with a greater freedom from reoperation/reintervention (68% ± 6.8%) when compared with tetralogy of Fallot/pulmonary atresia (5.3% ± 4.3%, P = .0001), tetralogy of Fallot/absent pulmonary valve (0%, P = .00315), or truncus arteriosus (4.2% ± 2.8%, P = .0001). Eight patients (4 with tetralogy of Fallot/pulmonary stenosis, 3 with tetralogy of Fallot/pulmonary atresia, and 1 with tetralogy of Fallot/absent valve) underwent placement of a transannular patch with monocusp valve. Among this group, freedom from reoperation/reintervention is 41.7% ± 20.5% at 2.5 years. Monocusp function, as determined by means of echocardiographic analysis obtained at 11.4 ± 11.7 months (range, 0.3–31 months) showed an average monocusp gradient of 23.5 ± 26.1 mm Hg, and 3 (37.5%) patients had more than moderate pulmonary regurgitation.ConclusionsThe durability of neonatal right ventricular outflow tract reconstruction is diagnosis and method dependent. Anatomy allowing right ventricular outflow tract patching (either transannular or nontransannular) provides a durability advantage compared with that seen with a homograft. There was no difference in performance between aortic and pulmonary homografts, and the monocusp valve has limited durability and effectiveness in neonatal right ventricular outflow tract surgery. The long-term outcomes of transannular and nontransannular patching techniques for neonatal repair of tetralogy of Fallot/pulmonary stenosis are similar.

ObjectivesTricuspid regurgitation (TR) remains a risk factor for morbidity and mortality through staged palliation in patients with hypoplastic left heart syndrome (HLHS). Reports on the mechanisms associated with TR in patients with HLHS are limited. Thus, we sought to describe our experience with tricuspid valve (TV) repair in these patients, focusing on the mechanisms of TR and corresponding surgical techniques.MethodsWe performed a retrospective single-center review (January 2000 to December 2012) of patients with HLHS undergoing TV repair and completing Fontan circulation. We evaluated the pre- and postoperative echocardiograms, intraoperative findings, and surgical techniques used.ResultsA total of 53 TV repairs were performed in 35 patients with HLHS completing staged palliation. TV repairs were performed at stage II in 15, between stage II and III in 4, at stage III in 27, and after stage III in 7. The surgical techniques for valvuloplasty included annuloplasty (38%), anteroseptal (AS) commissuroplasty (66%), anterior papillary muscle repositioning (11%), multiple commissuroplasties (9%), septal-posterior commissuroplasty (9%), and fenestration closure (4%). The predominant jet of TR emanated along the AS commissure in 68% of the cases. All patients survived the procedure and were discharged. Preoperative echocardiography showed a dilated TV annulus on the lateral dimension, anteroposterior dimension, and area that was significantly reduced after TV repair (P

ObjectivesPatients with congenitally corrected transposition of the great arteries can present with tricuspid regurgitation (TR) and Ebsteinoid dysplasia of the tricuspid valve. To determine the fate of the tricuspid valve after anatomic repair and the effectiveness of tricuspid valvuloplasty, we reviewed our experience with anatomic repair of congenitally corrected transposition of the great arteries.MethodsFrom 1992 to 2012, 106 patients with congenitally corrected transposition of the great arteries underwent anatomic repair. Of the 106 patients, 24 (22.6%) had moderate or greater TR before anatomic repair, 14 of whom had Ebsteinoid dysplasia. Nine patients (8.5%) had Ebsteinoid dysplasia without significant TR, and 73 patients (68.9%) had neither TR nor dysplasia.ResultsOf the 106 patients, 6 (5.6%) with TR underwent tricuspid valvuloplasty at anatomic repair, 5 with (21%) and 1 without (11%) Ebsteinoid dysplasia (P