Left ventricular dysfunction in repaired tetralogy of Fallot: incidence and impact on atrial arrhythmias at long term-follow up.

Left ventricle (LV) systolic dysfunction in repaired tetralogy of Fallot (TOF) has been identified as a risk factor for functional status and adverse outcome. The aims of this cross-sectional followed by a prospective study were: (1) to evaluate the prevalence of LV systolic dysfunction in a large cohort of adults with repaired tetralogy of Fallot, (2) to test the relationship between LV systolic dysfunction and other known risk factors and (3) to evaluate the impact of LV systolic dysfunction on adverse cardiac events. In a multicenter study, 237 adults repaired TOF (58 % males, age 30 ± 10 years) were evaluated by cardiac magnetic resonance (CMR). Demographics, surgical history, ECG, Echo-Color Doppler and follow-up data were recorded. LV was dilated (Z value >2) in 16 patients (6 %), however 56 patients (23.6 %) had a reduced LV systolic function left ventricle ejection fraction (LVEF) (Z value <-2). Patients with LV systolic dysfunction were mainly males (82 %), had reduced right ventricle ejection fraction (RVEF), and higher right and left Late Gadolinium Enhanced scores. In a multivariate regression analysis male gender and RVEF resulted to be independent factors associated to LV systolic dysfunction. Atrial arrhythmias were the main adverse cardiac event at the follow-up and were associated to higher biventricular volumes and lower biventricular ejection fraction (EF); however multivariable analysis identified age, right ventricle end-diastolic volume (RVEDVi) and tricuspid regurgitation as independents factors associated to atrial arrhythmias. At long term follow-up at least » of repaired TOF has LV dysfunction. Lower LVEF is associated to male gender and lower RVEF.

Clinical Profile and Quality of Life of Adult Patients After the Fontan Procedure.

Increasingly, more patients with univentricular heart reach adulthood. Therefore, long-term psychological features are an important concern. The aim of this study was to evaluate the clinical and psychological profile of post-Fontan adult patients and to identify the most significant determinants of quality of life. In this retrospective cross-sectional study, we reviewed the surgical and medical history of post-Fontan adult patients. Patients underwent a 24-h electrocardiogram, echocardiography and exercise testing. Self-report questionnaires were used to assess the Work Ability Index, quality of life (Satisfaction with Life Scale), perceived health status (SF-36 questionnaire), coping strategies (Brief Cope questionnaire) and presence of mood disorders (Hospital Anxiety and Depression Scale). Thirty-nine patients aged between 18 and 48 years (mean 27.5 years) were enrolled. The mean follow-up was 21.5 years. Most patients were unmarried (82.9 %), had a high school diploma (62.9 %) and were employed (62.9 %). Twenty-nine patients (82.3 %) had at least one long-term complication. The median single ventricle ejection fraction was 57 %, and the median maximal oxygen consumption was 26.8 ml/min/kg. This population tended to be anxious and to use adaptive coping strategies. Quality of life was perceived as excellent or good in 57.2 % of cases and was not related to either cardiac function or exercise capacity. Both quality of life and SF-36 domains were related to the Work Ability Index. This cohort of post-Fontan adult patients enjoyed a good quality of life irrespective of disease severity.

Changed outcomes of fetuses with congenital heart disease: new Italian Multicentre study.

OBJECTIVES: To analyse the outcomes of fetuses with congenital heart disease between 2000 and 2005 in comparison to a previous multicentre study regarding the period 1983-1996. PARTICIPANTS AND METHODS: Data of seven centres were prospectively collected, the inclusion criteria being a confirmed fetal diagnosis after birth or at autopsy and a known follow-up, for at least 6 months after birth. Data of 649 fetuses, median age at diagnosis 24 weeks' gestation (15-37), 340/649 (52.4%) diagnosed before 24 weeks, were analysed. RESULTS: Sixty seven and 59 cases had chromosomal or extracardiac anomalies (10.3 and 9.1%). Termination of pregnancy was chosen in 21.6% of cases versus 28.9% in the previous study, being significantly lower in cases with early diagnosis (P <0.001). Out of 509 fetuses continuing pregnancy, 23 died in utero (4.5%) and 110 (21.1%) postnatally, versus 43% in the previous study (P <0.0017). Total surgical/postprocedure death occurred in 20.6% (59/287 infants) versus 37% previously (P <0.003), 67 infants being premature and 35 with associated chromosomal or extracardiac anomalies. The current overall mortality rate was higher in cases with chromosomal or extracardiac anomalies (59.5 and 51.35%, respectively, whereas it was 20.1% in isolated congenital heart disease). Overall current survival was 376 of 509 (73.9%) versus 45% in the previous study (P <0.0001). CONCLUSION: Our data show a reduced overall and surgical mortality, with respect to our previous study, resulting from resulting from an improved perinatal management and treatment of affected fetuses in the more recent era.

Cardiac resynchronisation therapy associated with pulmonary artery banding in an adult with severe right ventricular dysfunction after Mustard repair for complete transposition of the great arteries: results after 2 years of follow-up.

Late dysfunction of the systemic right ventricle in patients with complete transposition of the great arteries after Mustard or Senning procedures and progressive deterioration of the clinical status has been demonstrated. However, evidence-based data on the effective therapy for systemic right ventricular dysfunction in these patients are yet to be defined. Our patient shows an improvement in the right ventricular systolic function, with a reduction in tricuspid regurgitation and a consequent better exercise tolerance after a hybrid approach consisting of an upgrading of a previous transvenous-implanted dual-chamber Implantable Cardiac Defibrillator to biventricular pacing associated with pulmonary artery banding via an anterior thoracotomy.

Heart failure in pregnant women with cardiac disease: data from the ROPAC.

OBJECTIVE: Heart failure (HF) is one of the most important complications in pregnant women with heart disease, causing maternal and fetal mortality and morbidity. METHODS: This is an international observational registry of patients with structural heart disease during pregnancy. Sixty hospitals in 28 countries enrolled 1321 women between 2007 and 2011. Pregnant women with valvular heart disease, congenital heart disease, ischaemic heart disease, or cardiomyopathy could be included. Main outcome measures were onset and predictors of HF and maternal and fetal death. RESULTS: In total, 173 (13.1%) of the 1321 patients developed HF, making HF the most common major cardiovascular complication during pregnancy. Baseline parameters associated with HF were New York Heart Association class ≥ 3, signs of HF, WHO category ≥ 3, cardiomyopathy or pulmonary hypertension. HF occurred at a median time of 31 weeks gestation (IQR 23-40) with the highest incidence at the end of the second trimester (34%) or peripartum (31%). Maternal mortality was higher in patients with HF (4.8% in patients with HF and 0.5% in those without HF p<0.001). Pre-eclampsia was strongly related to HF (OR 7.1, 95% CI 3.9 to 13.2, p<0.001). Fetal death and the incidence of preterm birth were higher in women with HF compared to women without HF (4.6% vs 1.2%, p=0.001; and 30% vs 13%, p=0.001). CONCLUSIONS: HF was the most common complication during pregnancy, and occurred typically at the end of the second trimester, or after birth. It was most common in women with cardiomyopathy or pulmonary hypertension and was strongly associated with pre-eclampsia and an adverse maternal and perinatal outcome.

Long-term results of treatment with bosentan in adult Eisenmenger's syndrome patients with Down's syndrome related to congenital heart disease.

BACKGROUND: Patients with Down's syndrome and shunt lesions are at high risk of developing pulmonary arterial hypertension (PAH) earlier than patients without Down's syndrome. However, data on the efficacy of PAH-specific therapy in patients with Down's syndrome are limited. The aim of this retrospective analysis was to determine the long-term efficacy of the dual endothelin receptor antagonist, bosentan, in Eisenmenger's syndrome (ES) patients with Down's syndrome. METHODS: In this observational study adults with Down's syndrome with a confirmed diagnosis of ES (World Health Organization functional class III) and receiving bosentan therapy and were followed up long term. Clinical evaluation at baseline and follow-up visits included resting transcutaneous arterial oxygen saturation and laboratory assessments. Exercise capacity was evaluated using a 6-minute walk test where transcutaneous arterial oxygen saturation at peak exercise (SpO2), 6-minute walk distance (6MWD) and Borg dyspnoea index were assessed. A full echocardiographic assessment was conducted at baseline and follow-up visits. RESULTS: Overall, seven adults (mean age 29.6 ± 11.2 years; 57% male) received bosentan at a starting dose of 62.5 mg twice daily. This was increased to the target dose of 125 mg twice daily 4 weeks later. All patients remained on bosentan until the end of the study. After a mean (± standard deviation) duration of 52.2 ± 3.9 months (range: 46.0-55.5 months), 6MWD had increased from 199.6 ± 69.1 metres to 303.7 ± 99.9 metres (P < 0.05) and SpO2 at the end of the 6-minute walk test had increased from 61.6 ± 7.6% to 74.7 ± 6.2% (P < 0.05). Echocardiography demonstrated a significant change in acceleration time from 62.9 ± 11.6 m/s to 83.0 ± 9.6 m/s (P = 0.0156), and acceleration time/ejection time ratio from the pulmonary flow from 0.24 ± 0.04 at baseline to 0.30 ± 0.02 (P = 0.0156) at final follow-up. CONCLUSIONS: Long-term treatment with bosentan significantly improved exercise capacity and oxygen saturation following exercise in adult ES patients with Down's syndrome. These data confirm that the presence of Down's syndrome does not affect the response to oral bosentan therapy.

Stenting of aortic coarctation and exercise-induced hypertension in the young.

OBJECTIVES: To evaluate whether the stenting of aortic coarctation enhance the risk of exercise-induced hypertension (EIH). BACKGROUND: There is the theoretical concern that aortic stents may cause increased aortic wall impedance and therefore systolic hypertension during exercise. METHODS: Blood pressure and the Doppler derived peak and mean systolic pressure gradient (PSG and MSG) across the distal aorta at the peak of exercise were evaluated in young patients (mean age 14 +/- 3 years) with aortic coarctation successfully treated with surgery or with stent implantation at least 1 year before the test. Only patients who reached the 85% maximal predicted heart rate or whose exercise test was interrupted because of severe hypertension, and in whom significant aortic narrowings were excluded by a MRI or a CT scan performed in the six months preceding the exercise test were included in the study. RESULTS: Seventeen patients formed the surgery-group, while 15 patients the stent-group. Patients in surgery-group were younger at coarctation repair and with a longer follow-up than those in stent-group. No difference was present regarding age, body surface area, gender, and presence, and degree of mildly hypoplastic aortic segments between the two groups as well as between patients with or without EIH. EIH was found in 35% of surgery-group patients and in 33% of stent-group patients. PSG and MSG were similar in the patients with or without EIH. CONCLUSIONS: EIH can be found in a high number of young patients successfully treated for aortic coarctation but at intermediate follow-up stent implantation does not seem to enhance the risk of EIH.

Age at repair affects the very long-term outcome of sinus venosus defect.

BACKGROUND: Records of patients who had repair of sinus venosus defect (SVD) between 1970 and 2008 were reviewed to predict very long-term outcome. METHODS: Repairs occurred in 104 consecutive patients (51 men), aged 29 +/- 23 years (range, 1 to 70 years). Seven had isolated SVD and 97 had associated lesions that required concomitant operations. Five patients had preoperative arrhythmias; 24 (23%) were in New York Heart Association (NYHA) class III to V. Single-patch repair was done in 91 patients, caval translocation (Warden) in 7, and double-patch in 6. RESULTS: Ten late deaths during 38 years of follow-up (mean, 15 +/- 20 years). Survival was 97% +/- 2% and 79% +/- 7% at 10 and 30 years. Thirty-one (29%) long-term survivors experienced 47 complications, including chronic/recurrent supraventricular tachycardia in 28, heart failure in 5, permanent pacing in 8, cerebrovascular accident in 3, and unrelated cardiac reoperation in 3. At 30 years, freedom from adverse cardiac events was 47% +/- 9%, from supraventricular tachycardia, 50% +/- 9%; from permanent pacing, 83 +/- 6%; and from cerebrovascular accident, 96% +/- 2%. Follow-up age was 42 +/- 23 years (range, 5 to 82 years); 74 patients (79%) were in NYHA class I, and 15 and 5 were in class II and III to IV, respectively. Baseline cardiac rhythm was sinus in 75 patients (84%), atrial fibrillation in 11 (12%), and paced in 8. Nine patients had moderate/severe pulmonary hypertension, and 8 had left ventricular dysfunction. Only older age at operation was associated with lower survival (p = 0.003), freedom from cardiac events (p = 0.001), supraventricular tachycardia (p = 0.009), and permanent pacing (p = 0.002). Repair before age 20 was associated with lower NYHA class at follow-up (p = 0.01). CONCLUSIONS: SVD repair at an older age is associated with increased risk of late mortality, adverse cardiac events, and worse functional outcome. Repair during childhood is strongly advised.

Age-related variations of left ventricular endocardial and midwall function in healthy infants, children, and adolescents.

BACKGROUND: In pediatric age echocardiographic evaluation of left ventricular systolic function is usually based on indexes obtained by measurements at the endocardial level. In the presence of ventricular hypertrophy this may lead to an overestimation of systolic function. The aim of this study was to assess the developmental changes of left ventricular systolic mechanics measured at the endocardial and midwall levels. METHODS: In 239 normal subjects divided into six age groups we measured left ventricular end-diastolic volume, mass and mass/volume ratio, fractional shortening, and rate-corrected mean velocity of circumferential shortening at the endocardial and midwall levels. Endocardial meridional end-systolic stress and midwall circumferential end-systolic stress were considered as indexes of afterload. Relations of extent and velocity of fiber shortening to afterload at the endocardial and midwall levels were used to assess left ventricular contractility. RESULTS: Blood pressure, left ventricular afterload, volume and mass increased, whereas the mass/volume ratio remained stable during growth. Fractional shortening and mean velocity of circumferential shortening at the endocardial level decreased and showed an inverse relation to afterload. Midwall fractional shortening and rate-corrected mean velocity of circumferential shortening were lower during the first months and did not change during the first year of life. CONCLUSIONS: Left ventricular volume and mass increase with age, mass/volume ratio remains almost constant while afterload increases. Endocardial systolic function indexes are higher in the first period of life, due to low afterload and increased mass/volume ratio. In the first months of life the left ventricular myocardium shows a greater sensitivity to changes in afterload and a reduced contractility measured at the midwall level.

Factors affecting left ventricular remodelling and mechanics in the long-term follow-up after successful repair of aortic coarctation.

AIMS: To identify factors predisposing to abnormal left ventricular geometry and mechanics in 52 patients after successful repair of aortic coarctation. METHODS AND RESULTS: We evaluated left ventricular remodelling, systolic midwall mechanics, and isthmic gradient by echo-Doppler, systemic blood pressure at rest/exercise and by ambulatory blood pressure monitoring, and the aortic arch by magnetic resonance imaging. Echocardiographic findings were compared with those of 142 controls. The patients with aortic coarctation showed an increased indexed left ventricular end-diastolic volume, increased mass index, increased ratio of mass to volume and systolic chamber function. The contractility, estimated at midwall level, was increased in 21 percent of the patients. In 26 (50 percent) of the patients, we found abnormal left ventricular geometry, with 9 percent showing concentric remodelling, 33 percent eccentric hypertrophy, and 8 percent concentric hypertrophy. These patients were found to be older, underwent a later surgical repair, and to have higher systolic blood pressures at rest and exercise as well as during ambulatory monitoring. The relative mural thickness and mass index of the left ventricle showed a significant correlation with different variables on uni- and multivariate analysis. Age and diastolic blood pressure at rest are the only factors associated with abnormal left ventricular remodelling. CONCLUSIONS: Patients who have undergone a seemingly successful surgical repair of aortic coarctation may have persistently abnormal geometry with a hyperdynamic state of the left ventricle. This is more frequent in older patients, and in those with higher diastolic blood pressures.

Left ventricular remodeling, systolic function, and diastolic function in young adults with beta-thalassemia intermedia: a Doppler echocardiography study.

BACKGROUND: The aim of this study was to investigate the left ventricular (LV) remodeling and function in 24 asymptomatic young adults affected by beta-thalassemia intermedia (TI), in order to compare the obtained data with that of 80 patients affected by beta-thalassemia major (TM) and 65 healthy subjects. METHODS: LV volumes and shapes, mass index, mass/volume ratio, systolic and diastolic function, stroke volume, and cardiac index were determined by two-dimensional and M-mode echocardiography. RESULTS: In the TM and TI groups, LV volumes, diastolic and systolic shapes were significantly different from the control subjects, but the ejection fraction was slightly reduced only in the TM group. The TI group had larger LV volumes than did the TM group (mean [+/- SD] end-diastolic volume index, 99.4 +/- 21.9 vs 82.7 +/- 21.5 mL/m(2), respectively [p < 0.005]; mean end-systolic volume index, 42.8 +/- 12.2 vs 36.1 +/- 12.9 mL/m(2), respectively [p < 0.05]). Both groups showed an increase of the LV mass index, but the mass/volume ratio did not differ from the control subjects. The systolic volume index and the cardiac index were increased in both groups, but the increase was more pronounced in the TI group. Fractional shortening (FS) and the mean velocity of circumferential shortening (mVCFc) were decreased in the TM group (FS, 33.6 +/- 5.5% vs 36.9 +/- 4.1, respectively [p < 0.001]; mVCFc, 1.06 +/- 0.18 vs 1.17 +/- 0.12 circumference per second, respectively [p < 0.0001]). The LV contractile state was depressed only in the TM group, and the preload index was normal in both. LV filling showed an increase in the total flow velocity integral due to increases in the peak E wave (E) and peak A wave (A) velocities and integrals, with an increase of the E/A ratio in the TM group and a slight decrease in the TI group. The isovolumic relaxation time was prolonged in both groups. There was no major derangement in the pulmonary venous flow. CONCLUSIONS: Asymptomatic young adults with TI show significant increases in LV volumes, LV mass, and cardiac index that are more pronounced than those in TM patients. LV systolic function is preserved in the TI group but is slightly depressed in the TM group due to the increase of afterload and to reduced contractility. The hemodynamic and hematologic factors involved in the etiopathogenesis of these findings are discussed, such as the treatment strategy.

Systolic and diastolic performance late after the Fontan procedure for a single ventricle and comparison of those undergoing operation at <12 months of age and at >12 months of age.

To evaluate whether surgical history can influence systolic and diastolic properties of a functional single left ventricle after Fontan operation, we echocardiographically investigated 31 patients (mean age 93.7 months; range 21 to 276); 21 patients were >12 months of age (group A) and 10 were <12 months of age (group B) at the time of the Fontan and/or cavopulmonary procedure. In group A we found persistent abnormalities of left ventricular mass index (95.9 vs 64.1 g/m(2), p <0.05) at long-term follow-up, whereas group B had normal left ventricular mass (61.9 vs 64.1 g/m2, p = NS). In contrast, a diastolic pattern characterized by augmented late diastolic filling was present in both patient groups regardless of age at operation and length of follow-up (E/A in group A 1.3 +/- 0.4, E/A in group B 1.6 +/- 1.5, E/A in controls 1.7 +/- 0.6; A vs B, p = NS; A vs controls, p <0.05). We concluded that patients with a single left ventricle who undergo an unloading procedure performed within the first year of life have complete normalization of left ventricular mass, although a diastolic filling pattern suggestive of augmented compliance persists, regardless of the age at operation.