Multimodality imaging, single center, cross-sectional study in adolescents or young adults with repaired tetralogy of Fallout.

BACKGROUND: Proper integration of multiple imaging modalities in the routine follow-up of patients with repaired tetralogy of Fallout (TOF) is poorly supported by data. We report our single center comparative study between cardiac magnetic resonance (CMR) and echocardiography to assess equipoise in the clinical utility of these two imaging methods in an unselected consecutive cohort of TOF patients referred to our outpatient clinic. MATERIAL AND METHODS: In this cross-sectional study, repaired TOF patients who underwent CMR and echocardiography within a 4-week period between 2010 and 2011 at our Center were included. Linear regression was used to analyze degree of inter modality correlation. A prediction model tested the association between functional data/probrain natriuretic peptide (Pro-BNP) with CMR. RESULTS: Fifty patients were included in the study (mean age 31 ±â€Š18 years). The best predictors of right ventricle (RV) ejection fraction at CMR were tricuspid anular plane systolic excursion (tricuspid valve anular plane systolic excursion, R 0.37, P < 0.0001) and RV peak S-wave velocity (R 0.40, P < 0.001). Pro-BNP levels did present weak correlation with New York Heart Association functional class (R 0.31, P < 0.002) and QRS duration (R 0.32, P < 0.002) and a moderate correlation with right atrium area at CMR (R 0.46, P < 0.0001). CONCLUSION: We found limited correlation between the two imaging modalities in the evaluation of RV after intracardiac repair of TOF. Pro-BNP level presents moderate correlation with right atrium area measured with echocardiography. Serial CMR evaluations are needed in this patient population, but they may be interchanged by routine echocardiography in particular in patients with normal or stable echocardiographic parameters.

Diagnostic Accuracy of Aortic Root Cross-sectional Area/Height Ratio in Children and Young Adults with Marfan and Loeys-Dietz Syndrome.

BACKGROUND: Accurate quantification of aortic dilatation is critical in children with syndromes associated with thoracic aortic aneurysm, yet classification of normality is difficult. Current methods of normalization use body surface area to account for growth, despite a nonlinear relationship of body surface area to aortic root dimensions. In contrast, height has a linear relationship with aortic root dimensions in normal children, is simple to measure and requires no secondary calculation. We evaluated the diagnostic accuracy of an height-based aortic root-indexing method, aortic root cross-sectional area/height ratio (AHr), in children with Marfan and Loeys-Dietz syndromes. METHODS: A cohort of 54 children with Marfan or Loeys-Dietz syndromes, aged 3 months to 17 years, were evaluated with a transthoracic echocardiogram. AHr was measured in diastole at sinuses of Valsalva (SoV) and proximal ascending aorta (pAA) in a group of normal subjects matched for age and body surface area and normal values were provided. AHr values were recorded for patients and compared with z-scores results obtained with Gautier's and Campens's nomograms. RESULTS: AHr values in the group of normal subjects were 2.6 ± 0.6 at SoV and 2 ± 0.5 at pAA. Categorization of z-scores and AHr showed good correspondence between AHr and Gautier's method (P = .341 at SoV and .185 at pAA) and AHr and Campens method (P =.465 at SoV and 0.110 at pAA). CONCLUSIONS: There was a good correspondence of AHr results with two different z-scores. AHr is a simple to use and valid option to quantify aortic root dilatation in pediatric patients.

[Aortic root dilatation rate in pediatric patients with Marfan syndrome treated with losartan]. / Progressione della dilatazione aortica nel paziente pediatrico con sindrome di Marfan in terapia medica con losartan.

BACKGROUND: Medical therapy with angiotensin II receptor blockers/angiotensin-converting enzyme inhibitors and/or beta-blockers was reported to reduce aortic root dilatation rates in pediatric patients with Marfan syndrome. No data are available in the literature on losartan effects after 3 years of therapy. The aim of our study was to establish whether losartan reduces aortic root dilatation rates in pediatric patients with Marfan syndrome in the mid and long term. METHODS: This is a retrospective analysis of 38 pediatric patients with Marfan syndrome followed at the Marfan Clinic of S. Orsola-Malpighi Hospital of the University of Bologna (Italy). Aortic diameters were measured at sinuses of Valsalva and proximal ascending aorta with transthoracic echocardiography. RESULTS: After a mean follow-up of 4.5 ± 2.5 years (range 2-9 years), aortic root z score at sinuses of Valsalva and proximal ascending aorta remained stable. The average annual rate of change in aortic root z score was -0.1 ± 0.4 and 0 ± 0.3 at sinuses of Valsalva and proximal ascending aorta, respectively. The mean dose of losartan was 0.7 ± 0.3 mg/kg/day. Three patients were non-responders, probably because of late beginning or low dose of therapy. Eight patients underwent cardiac surgery (aortic root surgery in 5 and mitral valve repair in 3), all of them started losartan later in life. CONCLUSIONS: Despite the retrospective design of the study and the small sample size, a beneficial effect of losartan therapy was observed in pediatric patients with Marfan syndrome in the mid and long term. Late beginning or low doses of losartan can turn off the effects of therapy.

Rasmussen's aneurysm in childhood: a case report.

In medical literature, few cases of Rasmussen's aneurysm have been reported until now, and to the best of our knowledge, there has been no description of a case of Rasmussen's aneurysm in childhood. The child described in the case report had a restrictive cardiomyopathy, complicated by pulmonary hypertension, in association with miliary tuberculosis.

Exercise training improves exercise capacity in adult patients with a systemic right ventricle: a randomized clinical trial.

OBJECTIVE: To assess whether exercise training in adult patients with a systemic right ventricle (RV) improves exercise capacity and quality of life and lowers serum N-terminal prohormone brain natriuretic peptide (NT-proBNP) levels. DESIGN: Multi-centre parallel randomized controlled trial. PARTICIPANTS: Patients with a systemic RV due to congenitally or surgically corrected transposition of the great arteries. METHODS: Fifty-four adult patients with a systemic RV, were randomized using unmarked opaque envelopes to an intervention group (n = 28) with three training sessions per week for 10 consecutive weeks, and a control group (n = 26). Randomization was stratified by participating centre. At baseline, and follow-up, we determined maximal exercise capacity (V'O(2peak)), serum NT-proBNP levels, and quality of life by means of the SF-36, and the TAAQOL Congenital Heart Disease questionnaires. The final analysis was performed by linear regression, taking into account the stratified randomization. RESULTS: Forty-six patients were analysed (male 50%, age 32 ± 11 years, intervention group n = 24, control group n = 22). Analysis at 10 weeks showed a significant difference in V'O(2peak) (3.4 mL/kg/min, 95% CI: 0.2 to 6.7; P = 0.04) and resting systolic blood pressure (-7.6 mmHg, 95% CI: -14.0 to -1.3; P = 0.03) in favour of the exercise group. No significant changes were found in serum NT-proBNP levels or quality of life in the intervention group or in the control group nor between groups. None of the patients in the intervention group had to discontinue the training programme due to adverse events. CONCLUSION: In adult patients with a systemic RV exercise training improve exercise capacity. We recommend to revise restrictive guidelines, and to encourage patients to become physically active. ( TRIAL REGISTRATION: The study was registered at http://trialregister.nl. Identifier: NTR1909.).

[Results and complications of permanent pacing in pediatric patients with congenital or acquired atrioventricular block]. / Risultati e complicanze dell'impianto di pacemaker definitivo in pazienti pediatrici con blocco atrioventricolare congenito ed acquisito.

BACKGROUND: Currently there is no evidence to prefer an endocardial or epicardial approach for pacing in pediatric patients. This analysis was aimed at defining the complications of pacemaker implantation in a pediatric population with atrioventricular block according to a strategy of choosing an epicardial system for patients <10 kg and an endocardial system for patients >10 kg. METHODS: This is a retrospective study performed on 27 patients, with and without congenital heart disease, implanted with a permanent pacemaker because of idiopathic or acquired atrioventricular block at our Pediatric Cardiology and Cardiac Surgery Unit of S. Orsola-Malpighi Hospital in Bologna (Italy) between 1981 and 2010. Patients were divided into two groups: 70% of the population (group A) was implanted with an epicardial system, 30% (group B) with an endocardial system. RESULTS: After a mean follow-up of 14 years (17 ± 8 years for group A, 7 ± 5 years for group B), the most frequent complications were lead failure (44%) and infection (18%). Both were more common in the epicardial system group: lead failure rate 53% in group A vs 25% in group B (p=0.0001); infection rate 26% in group A vs 0% in group B (p=0.0001); rate of system revision or implantation of a new electrode 42% in group A vs 12% in group B (p=0.0001). CONCLUSIONS: The present study demonstrates a high complication rate in patients undergoing an epicardial pacing system implantation. However, in patients <10 kg the epicardial system allows subclavian venous access protection for endocardial system implantation after somatic growth, avoiding high-risk procedures of system revision or lead extraction and subclavian vein occlusion in the long term.

Dilated cardiomyopathy presenting in childhood: aetiology, diagnostic approach, and clinical course.

OBJECTIVE: To determine the outcome of dilated cardiomyopathy presenting in childhood and the features that might be useful for prognostic stratification. METHODS: Retrospective study of 41 consecutive children affected by dilated cardiomyopathy - aged 0-14 years; median 33.4 plus or minus 49.25 - between 1993 and 2008. We reviewed the medical history to determine age at diagnosis, family history, previous viral illness, aetiology, symptoms and signs at presentation, treatment, and outcome. The diagnosis was made on the basis of cardiomegaly and evidence of poor left ventricular function by echocardiography. We also carried out a metabolic evaluation including blood lactate, pyruvate, carnitine, amino acids, urine organic acids, assessment of respiratory chain enzymes, and analysis of histopathological material. Survival curves were constructed by the Kaplan-Meier method. RESULTS: Follow-up ranged from 10 days to 162 months - median 45.25 plus or minus 41.15 months. Freedom from death or cardiac transplantation was 68.3% at 5 years. The primary end-point of death/cardiac transplantation was associated with the need for intravenous inotropic support. A trend towards a poorer prognosis was found for age at diagnosis of more than 5 years and for a metabolic aetiology of dilated cardiomyopathy. For the children affected by cardiomyopathy as part of a multi-system involvement, mortality was 50%. CONCLUSIONS: In children, dilated cardiomyopathy is a diverse disorder with outcomes that depend on cause, age, and cardiac failure status at presentation. Overt cardiac failure at presentation is a major prognostic factor for death or cardiac transplantation. Older age at presentation and metabolic aetiology may be associated with a poorer prognosis.

Long-term coronary artery outcome after arterial switch operation for transposition of the great arteries.

OBJECTIVE: To analyse the long-term patency of coronary arteries after neonatal arterial switch operation (ASO). METHODS: A retrospective study of the operative reports, follow-up and postoperative catheterisation data of 119 patients, who underwent the great arteries (TGA) repair since 1991, has been carried out. PATIENT POPULATION: Among the 133 survivors of the 137 ASOs performed between 1991 and 2007, 119 patients have been studied by routine control cardiac catheterisation and form the study population. Median time between repair and the coronary angiography was 2.9±1.9 years. A comparison between the eight patients (6.7% out of the entire study population), known to have postoperative coronary obstructions (group I) and the rest of the cohort with angiographic normal coronary vessels (group II) was performed by univariate analysis of variance and logistic regression models. One patient had surgical plasty of the left coronary main stem with subsequent percutaneous angioplasty, three patients had primary coronary stent implantation and four patients had no further intervention at all. In group I, all but one patient denied symptoms of chest pain and echocardiography failed to show any difference between the two groups in terms of left ventricular systolic function (ejection fraction group I 61±2% vs 62±6% of group II, p=1.0). RESULTS: The association of coronary obstruction with complex native coronary anatomy (Yacoub type B to E) was evident at both univariate (62% of group I vs 22% of group II, p=0.04) and logistic regression (p=0.007, odds ratio (OR) 8.1) models. The type of coronary reimplantation (i.e., coronary buttons on punch vs trap-door techniques) was similar between the two groups (punch reimplantation in 25% of patients of group I vs 31% of group II, p=0.1) as was the relative position of the great vessels (aorta anterior in 100% of patients of group I vs 96% of group II; univariate, p=0.1). CONCLUSIONS: The late outcome in terms of survival and functional status after ASO is excellent. Nevertheless, the risk of a clinically silent late coronary artery obstruction of the reimplanted coronary arteries warrants a prolonged follow-up protocol involving invasive angiographic assessment.

Are nasal decongestants safer than rhinitis? A case of oxymetazoline-induced syncope.

Derivatives of Imidazoline usually act to stimulate peripheral alpha2 receptors causing vasoconstriction. In young children, however, they can also stimulate alpha2receptors in the cardiovascular and central nervous systems, possibly causing cardiovascular, neurological, and respiratory depression. These medications do not require medical prescriptions, so often parents use them, bypassing paediatricians. We report here a case of cardiovascular and neurological depression induced by oxymetalzoline in a toddler.

Correlations of phenotype and genotype in relation to morphologic remodelling of the aortic root in patients with Turner's syndrome.

BACKGROUND: Patients with Turner's syndrome are at risk of aortic dilation and dissection. Currently, it is not known whether such dilation is related to associated cardiovascular abnormalities, or to the genetic anomaly itself. METHODS: We studied echocardiographically 107 patients with genetically proven Turner's syndrome, with heterogeneous underlying karyotypes, and without associated cardiac lesions. Their average age was 19.6 plus or minus 8.4 years. We compared the finding with those from 71 age-matched healthy female volunteers. The diameter of the aorta was measured at the level of the basal attachments of the aortic valvar leaflets, the sinuses of Valsalva, the sinutubular junction, and its ascending component. RESULTS: Compared to control subjects, the patients with Turner's syndrome had larger diameters of the aorta at the level of the sinuses of Valsalva, at 23.4+/-4.8 versus 25.5+/-4.1 millimetres (p = 0.0014), the sinutubular junction, at 19.9+/-3.8 versus 23.3+/-4.1 millimetres (p < 0.0001), and the ascending aorta, at 22.3+/-4.9 versus 24.6+/-4.4 millimetres (p = 0.0011). Dilation of the sinutubular junction, found in just over one-quarter of the patients, was more common than dilation of the ascending aorta, the latter found in less than one-tenth. The patients with Turner's syndrome, therefore, presented with remodelling of the aortic root, with relative dilation of the sinutubular junction. The underlying karyotype influenced both the dimensions of the sinutubular junction (p = 0.0054), and the ascending aorta (p = 0.0064), so that patients with the karyotype 45X had larger aortas. The karyotype was the strongest predictor by multivariate analysis for dilation at both these sites (p = 0.0138 and 0.0085, respectively). CONCLUSIONS: Dilation at the sinutubular junction is frequent in patients with Turner's syndrome, and is more common than dilation of the ascending aorta. The syndrome is associated with a remodelling of the aortic root, with prominent dilation of the sinutubular junction. There seems to be a relation between aortic dilation and the underlying genotype.

Ventilatory efficiency and aerobic capacity predict event-free survival in adults with atrial repair for complete transposition of the great arteries.

OBJECTIVES: The goal of this study was to assess the prognostic value of the cardiopulmonary exercise test (CPET) in patients who received a Mustard and Senning (M/S) operation. BACKGROUND: Patients who received an M/S operation have increased long-term risk of cardiovascular morbidity and mortality. Limited information is available on how to stratify risk in this population. METHODS: Between 1996 and 2007, 274 adults (age 26.3 +/- 8.9 years, range 16 to 50 years) who had received a Mustard (n = 144) or Senning (n = 130) operation in infancy were studied with CPET. During a follow-up of 3.9 +/- 2.3 years (range 0.2 to 10.8 years), 12 patients died at an age of 36 +/- 14 years, and 46 patients required a cardiac-related emergency (<24 h from the onset of symptom/condition) hospital admission at an age of 30 +/- 11 years. RESULTS: At multivariate Cox analysis, the slope of ventilation per unit of carbon dioxide output (VE/VCO(2) slope) (hazard ratio: 1.088, p < 0.0001) and percentage of predicted peak oxygen uptake (Vo(2)%) (hazard ratio: 0.979, p = 0.0136) were the strongest predictors of death/cardiac-related emergency hospital admission among demographic, clinical, and exercise variables. A VE/VCO(2) slope > or =35.4 (hazard ratio: 10.7, 95% confidence interval [CI]: 7.8 to 24.6), and a peak Vo(2)% < or =52.3% (hazard ratio: 3.4, 95% CI: 2.5 to 8.2) were associated with an increased 4-year risk of death/cardiac-related emergency hospital admission. Patients who had both a VE/VCO(2) slope > or =35.4 and a peak Vo(2)% < or =52.3% of predicted value were at highest risk (4-year event rate: 78.8%). CONCLUSIONS: CPET provides important prognostic information in adults with M/S operation. Subjects with enhanced ventilatory response to exercise or those with poor exercise capacity have a substantially higher 4-year risk of death/cardiac-related emergency hospital admission.

Heart transplantation in infants with idiopathic hypertrophic cardiomyopathy.

Whereas it is well known that idiopathic HCM can present in newborns and infants, little information is available on HT in this very young age group. We report a series of 17 infants with idiopathic HCM, including two neonates with rapidly progressive severe HF for whom HT was necessary. When HF manifests in a newborn/infant with idiopathic HCM and extreme cavity size reduction, the possibility of a rapidly progressive clinical course should be anticipated and HT may become the only available therapeutic solution.

Accuracy of oxygen uptake efficiency slope in adults with congenital heart disease.

BACKGROUND: Given its linearity throughout exercise, oxygen uptake efficiency slope (OUES) obtained with a sub-maximal exercise is considered a reliable predictor of exercise capacity. We sought to assess the linearity of OUES across different exercise stages in adults with various forms of congenital heart disease. METHODS: Using cardiopulmonary exercise testing, we studied 23 patients after a Fontan operation, and 30 patients after atrial repair for complete transposition of the great arteries, at a mean age of 24+/-10 years. Thirty-five healthy volunteers were used as controls. OUES was calculated from 100% (OUES), the first 50% (OUES(50)), and the last 50% (OUES(50-100)) of the entire exercise duration. RESULTS: Peak oxygen uptake and OUES were reduced in Fontan patients when compared to atrial repair or control subjects (p<0.05). However, whereas in atrial repair and in control subjects OUES(50), OUES(50-100), and OUES appeared to be similar (p>0.05), in Fontan patients OUES(50) appeared to be lower than OUES(50-100) (1.38+/-0.46 vs. 1.78+/-0.51, p=0.01) and OUES (1.38+/-0.46 vs. 1.72+/-0.56, p=0.032). The difference between OUES(50) and OUES(50-100) appeared particularly large in cyanotic Fontan patients (1.40+/-0.42 vs. 1.93+/-0.68, p=0.001), whereas no difference was observed in Fontan patients with normal saturation (1.33+/-0.59 vs. 1.37+/-0.67, p=0.922). CONCLUSIONS: In cyanotic Fontan patients, OUES(50) differs substantially from OUES(50-100) and OUES. Therefore, OUES(50) is unable to predict maximal exercise capacity in this population.

Long-term incidence of atrial fibrillation and flutter after transcatheter atrial septal defect closure in adults.

BACKGROUND: The long-term risk of atrial fibrillation and flutter (AFF) after trascatheter atrial septal defects (ASD) closure in adults is unknown. METHODS: We studied 134 patients who have undergone transcatheter ASD closure at our institution at an age of > 18 years (mean age 39 +/- 16 years); Patients were followed-up for 4.8 +/- 2.7 years (range 0.8-9.6 years). We assessed the presence of AFF both before and after ASD closure using standard 12 lead ECGs or 24h ambulatory Holter monitors. RESULTS: 13 patients (10%) had documented AFF before the procedure (paroxysmal in 6, permanent in 7). Patients with AFF before the procedure were older (p < 0.0001), and had worse clinical condition (p = 0.0008). Patients without a history of AFF before the procedure and those who experienced paroxysmal AFF before the procedure had a very low annual risk (0%) of subsequent permanent AFF at long-term follow-up. Four patients with permanent AFF before the procedure (onset of < 12months) underwent electrical cardioversion immediately before ASD closure. Two of them (50%) are in sinus rhythm after 4.1 and 7.0 years, respectively. CONCLUSIONS: Transcatheter ASD closure performed in adults with no history of AFF or with a history of paroxysmal AFF before closure seems to protect from development of AFF in the long-term. In selected patients with permanent AFF at closure, device ASD closure together with arrhythmia cardioversion might be able to restore and maintain sinus rhythm in the long-term.

Management of pulmonary arterial hypertension associated with congenital systemic-to-pulmonary shunts and Eisenmenger's syndrome.

A large proportion of patients with congenital heart disease (CHD), in particular those with relevant systemic-to-pulmonary shunts, will develop pulmonary arterial hypertension (PAH) if left untreated. Persistent exposure of the pulmonary vasculature to increased blood flow, as well as increased pressure, may result in pulmonary obstructive arteriopathy, which leads to increased pulmonary vascular resistance that, if it approaches or exceeds systemic resistance, will result in shunt reversal. Eisenmenger's syndrome, the most advanced form of PAH associated with CHD, is defined as CHD with an initial large systemic-to-pulmonary shunt that induces severe pulmonary vascular disease and PAH, with resultant reversal of the shunt and central cyanosis. The histopathological and pathobiological changes seen in patients with PAH associated with congenital systemic-to-pulmonary shunts, such as endothelial dysfunction of the pulmonary vasculature, are considered similar to those observed in idiopathic or other associated forms of PAH. A pathological and pathophysiological classification of CHD with systemic-to-pulmonary shunt leading to PAH has been developed that includes specific characteristics, such as the type, dimensions and direction of the shunt, extracardiac abnormalities and repair status. A clinically oriented classification has also been proposed. The prevalence of PAH associated with congenital systemic-to-pulmonary shunts in Western countries has been estimated to range between 1.6 and 12.5 cases per million adults, with 25-50% of this population affected by Eisenmenger's syndrome. Clinically, Eisenmenger's syndrome presents with multiple organ involvement, with progressive deterioration of function over time. The signs and symptoms of Eisenmenger's syndrome in the advanced stages include central cyanosis, dyspnoea, fatigue, haemoptysis, syncope and right-sided heart failure. Survival of patients with Eisenmenger's syndrome is clearly less than that of the general population, but appears to be better than that of patients with idiopathic PAH in a comparable functional class. The treatment strategy for patients with PAH associated with congenital systemic-to-pulmonary shunts and, in particular, those with Eisenmenger's syndrome is based mainly on clinical experience rather than being evidence based. General measures include recommendations for physical activity, pregnancy, infections, air travel, exposure to high altitudes and elective surgery, and that psychological assistance be provided as necessary. Phlebotomies are required only when hyperviscosity of the blood is evident, usually when the haematocrit is >65%. The use of supplemental oxygen therapy is controversial and it should be used only in patients in whom it produces a consistent increase in arterial oxygen saturation. Oral anticoagulant treatment with warfarin can be initiated in patients with pulmonary artery thrombosis and absent, or only mild, haemoptysis. The following three classes of drugs targeting the correction of abnormalities in endothelial dysfunction have been approved recently for the treatment of PAH: (i) prostanoids; (ii) endothelin receptor antagonists; and (iii) phosphodiesterase-5 inhibitors. The efficacy and safety of these compounds have been confirmed in uncontrolled studies in patients with PAH associated with corrected and uncorrected congenital systemic-to-pulmonary shunts, as well as in patients with Eisenmenger's syndrome. One randomized controlled trial reported favourable short- and long-term outcomes of treatment with the orally active dual endothelin receptor antagonist bosentan in patients with Eisenmenger's syndrome. Lung transplantation with repair of the cardiac defect or combined heart-lung transplantation are options for Eisenmenger's syndrome patients with a poor prognosis. A treatment algorithm based on the one used in the treatment of PAH patients is proposed for patients with PAH associated with corrected and uncorrected congenital systemic-to-pulmonary shunts and Eisenmenger's syndrome.

Heart transplantation in hypertrophic cardiomyopathy.

Heart transplantation (HT) is the sole therapeutic option for selected patients with hypertrophic cardiomyopathy (HC) and refractory heart failure. However, the results of HT have not been systematically investigated in HC. We assessed the pathophysiologic profile of HT candidates and the outcome after transplantation in 307 patients with HC consecutively evaluated at our tertiary referral center from 1987 to 2005; follow-up was 9.9+8.2 years. Outcome of recipients with HC was compared with that of 141 patients who underwent transplantation for idiopathic dilated cardiomyopathy at our center over the same period. Of 21 patients with HC who entered the transplantation list, 20 had end-stage evolution with systolic dysfunction and 1 had an extremely small left ventricular cavity with impaired filling and recurrent cardiogenic shock during paroxysmal atrial fibrillation. Of 33 study patients with HC who showed end-stage evolution during follow-up, the 23 who were included on the waiting list or died from refractory heart failure (2 patients) were significantly younger than the 10 patients who remained clinically stable (37+/-14 vs 57+/-17 years, p=0.004). Of the 21 HT candidates, 18 underwent transplantation during follow-up. In heart transplant recipients, 7-year survival rate was 94% and not different from that of the 141 patients who received transplants for idiopathic dilated cardiomyopathy (p=0.66). In conclusion, long-term outcome after HT in patients with HC is favorable and similar to that of patients with idiopathic dilated cardiomyopathy. In patients with end-stage HC, young age is associated with more rapid progression to refractory heart failure.

Natural history of exercise capacity after the Fontan operation: a longitudinal study.

BACKGROUND: Previous studies have shown that older Fontan patients and those with an underlying morphologically right ventricle have lower exercise capacity. We sought to assess the natural history of exercise capacity after the Fontan operation in individual patients, and to identify the factors influencing the rate of decrease of exercise capacity over time. METHODS: We studied, longitudinally, 53 Fontan patients with cardiopulmonary exercise tests (average 3.2 +/- 1.1 tests for each patient). Age at the first test was 14 +/- 6 years. Time intervals between tests ranged from 1 to 16 years (average, 7.7 +/- 4.0 years). The morphology of the functionally single ventricle was left in 29 patients (55%) and right in 24 patients (45%). Twenty-two patients had undergone a total cavopulmonary connection (TCPC) at a mean age of 5.9 +/- 2.8 years. Thirty-one patients had undergone an atriopulmonary or an atrioventricular connection at an age of 6.9 +/- 4.4 years. Exercise capacity was expressed as percentage of predicted peak oxygen uptake (VO2). RESULTS: Overall, peak VO2 decreased at a rate of -2.6 +/- 2.7%/year. Ventricular morphology (r = 0.525, p = 0.0001) and type of Fontan operation (r = 0.381, p = 0.0057) appeared as the only predictors of the rate of decrease of peak VO2 at multivariate analysis. Patients with an underlying left ventricular morphology (-1.7 +/- 2.0 vs -3.7 +/- 3.2%/year, p = 0.005), and those who underwent a TCPC (-1.9 +/- 1.8 vs -3.3 +/- 3.2%/year, p = 0.042), had the lowest rate of decrease in peak VO2. CONCLUSIONS: Exercise capacity progressively declines in Fontan subjects. The decline of exercise capacity seems to be slower in patients with an underlying left ventricular morphology and in those who received a TCPC.

Long-term impact of transcatheter atrial septal defect closure in adults on cardiac function and exercise capacity.

BACKGROUND: The long-term impact of transcatheter atrial septal defect (ASD) closure on right ventricular (RV) remodeling and exercise capacity is unknown. METHODS: We studied with cardiopulmonary exercise testing and transthoracic echocardiography 29 adults (age 42.3+/-16.4 years) with hemodynamically significant ASD just before transcatheter defect closure and after 6 and >36 months from closure. RESULTS: Compared to 6 months after closure, a further improvement of peak oxygen uptake (p<0.001) and of the slope of ventilation/carbon dioxide production (p<0.001) was observed 3 years after the procedure, so that peak oxygen uptake appeared to be within the normal range in 23/29 patients (79%). Right ventricular short-axis (p<0.05) and long-axis (p<0.05) diameters further decreased beyond the 6-month period. The long-term improvement in exercise capacity correlated with pulmonary-to-systemic flow ratio (R=0.55, p=0.003) and with percentage decrease in RV short-axis diameter (R=0.59, p=0.002), but it did not correlate with age at closure (R=0.25, p=0.46). All patients who did not achieve a normal exercise capacity after 3 years from closure had a severely depressed pre-closure peak oxygen uptake (<50% of predicted). CONCLUSIONS: Adults who undergo transcatheter ASD closure may experience a further improvement in exercise capacity in the long term. The long-term improvement in exercise capacity is associated to an improvement in cardiac form and function and is not influenced by age at closure. Even if the majority of patients may reach a normal exercise capacity after ASD closure, an abnormal exercise capacity may persist in those patients that had a peak oxygen uptake below 50% of predicted value before the procedure.