Predicting Survival in Repaired Tetralogy of Fallot: A Lesion-Specific and Personalized Approach.

OBJECTIVES: This study sought to identify patients with repaired tetralogy of Fallot (rTOF) at high risk of death and malignant ventricular arrhythmia (VA). BACKGROUND: To date there is no robust risk stratification scheme to predict outcomes in adults with rTOF. METHODS: Consecutive patients were prospectively recruited for late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) to define right and left ventricular (RV, LV) fibrosis in addition to proven risk markers. RESULTS: The primary endpoint was all-cause mortality. Of the 550 patients (median age 32 years, 56% male), 27 died (mean follow-up 6.4 ± 5.8; total 3,512 years). Mortality was independently predicted by RVLGE extent, presence of LVLGE, RV ejection fraction ≤47%, LV ejection fraction ≤55%, B-type natriuretic peptide ≥127 ng/L, peak exercise oxygen uptake (V02) ≤17 mL/kg/min, prior sustained atrial arrhythmia, and age ≥50 years. The weighted scores for each of the preceding independent predictors differentiated a high-risk subgroup of patients with a 4.4%, annual risk of mortality (area under the curve [AUC]: 0.87; P < 0.001). The secondary endpoint (VA), a composite of life-threatening sustained ventricular tachycardia/resuscitated ventricular fibrillation/sudden cardiac death occurred in 29. Weighted scores that included several predictors of mortality and RV outflow tract akinetic length ≥55 mm and RV systolic pressure ≥47 mm Hg identified high-risk patients with a 3.7% annual risk of VA (AUC: 0.79; P < 0.001) RVLGE was heavily weighted in both risk scores caused by its strong relative prognostic value. CONCLUSIONS: We present a score integrating multiple appropriately weighted risk factors to identify the subgroup of patients with rTOF who are at high annual risk of death who may benefit from targeted therapy.

Does gender affect the prognosis and risk of complications in patients with congenital heart disease in the modern era?

Gender differences in the outcome of acquired cardiovascular disease are well known, but available literature on the influence of gender in congenital heart disease (CHD) is limited. Registries have provided valuable, albeit at times conflicting data. Higher mortality rates have been reported in older males with CHD, while sudden cardiac death is more prevalent in young males. However, mortality around surgery for CHD is higher in girls compared to boys, likely due to smaller body size. Women are at higher risk of developing pulmonary arterial hypertension, but at lower risk of adverse aortic outcomes, even though they are less likely to receive aortic surgery. Finally, women have a lower risk of presenting with infective endocarditis compared to men. The underlying reasons for gender differences in CHD can be attributed to genetic, hormonal, behavioural and other causes. The aim of the present paper is to provide an overview of available evidence on gender differences in CHD and their impact on outcome.

Clinical course and potential complications of small ventricular septal defects in adulthood: Late development of left ventricular dysfunction justifies lifelong care.

BACKGROUND: Patients with small ventricular septal defects (VSDs) are thought to have excellent long-term survival, although complications may not be uncommon. METHODS: We identified all patients aged ≥ 16 years with native isolated VSD between January 2000 and September 2013. Clinical outcomes were retrospectively reviewed. Transthoracic echocardiograms performed within 2 years of last assessment were reviewed for hemodynamic sequelae. RESULTS: Two-hundred-and-thirty-one patients, 100 (43%) male, mean age at last follow-up 34 ± 13 years were studied. During the recorded period there were no deaths. The majority (224/231; 97%) were asymptomatic. Documented arrhythmias occurred in 7 patients (3%), double-chamber right ventricle (DCRV) in 29 (13%), more than mild aortic regurgitation in 6 (3%) and infective endocarditis in 24 (10%) patients. Surgery due to complications associated with VSD was performed in 26 (11%) patients at a median age of 27.6 years (IQR: 16.1-38.7) due to DCRV (n=17, 65%), infective endocarditis (n=6, 23%), progression of left-right shunt (n=2, 8%) and aortic regurgitation (n=1, 4%). At most recent echocardiography (n=164), 10 (6%), had reduced LVEF, 34 (21%) had increased LVEDD and 17 (10%) had LVESD >4.0 cm. Thirty-two patients (25%) with normal LV dimensions had LA enlargement suggesting LV diastolic dysfunction. CONCLUSIONS: We report a non-negligible incidence of major complications or clinical events during late follow-up of adult patients with restrictive VSDs. Furthermore, we show co-existing LV dysfunction, systolic or diastolic in a subset of patients. Indication for VSD closure in childhood may be recognized, whereas lifelong follow-up for adult with restrictive VSDs is clearly warranted.

Right ventricular mechanics and QRS duration in patients with repaired tetralogy of Fallot: implications of infundibular disease.

BACKGROUND: Patients after repair of tetralogy of Fallot (ToF) frequently have right ventricular (RV) dysfunction and prolonged QRS duration (QRSd) and thus could be candidates for cardiac resynchronization therapy. We aimed to assess the relationship between QRSd and the timing of RV wall motion, including the RV outflow tract (RVOT), in these patients. METHODS AND RESULTS: Sixty-seven repaired ToF patients (median age, 34 years; interquartile range, 24 to 43 years) and 35 age-matched control subjects were studied by echocardiography and cardiovascular magnetic resonance (n=55 of 67 ToF patients). Time intervals of the RV cardiac cycle were measured from Doppler recordings. Long-axis M-mode recordings were acquired from the right ventricular (RV) free wall and RV outflow tract (RVOT), and the delay in onset of long-axis shortening was measured. ToF patients showed minor abnormalities of the RV cardiac cycle unrelated to QRSd. RV ejection time was prolonged and correspondingly filling time was reduced compared with control subjects (22.3+/-2.6 versus 20.0+/-2.9 s/min, P<0.0001; 29.0+/-3.8 versus 32.7+/-3.5 s/min, P<0.0001). Total isovolumic time was normal in ToF patients (8.7+/-4.0 versus 7.4+/-2.9 s/min; P=NS). QRSd correlated with the delay in RV free wall motion (r=0.55, P<0.0001) and more so with the delay in RVOT shortening (r=0.82, P<0.0001). QRSd also correlated with measures of RVOT abnormality such as long-axis RVOT excursion and akinetic area length (r=-0.46, P=0.004; r=0.33, P=0.01). CONCLUSIONS: QRSd in postoperative ToF patients reflects mainly abnormalities of the RVOT rather than the RV body itself. Thus, prevention and treatment of mechanical asynchrony and malignant arrhythmia should focus on the RV infundibulum. Indications for cardiac resynchronization therapy after ToF repair warrant further investigation.