This retrospective multicenter study represents an analysis of the intrauterine determinants of the prognosis for conotruncal anomalies. Data regarding reason for referral, presence of chromosomal or extracardiac anomalies, pregnancy and surgical outcome were recorded in 67 cases of conotruncal anomalies from three Italian referral units. Chromosomal aberrations effected 11 of the 60 (18.3%) fetuses in which a karyotype was available. Extra-cardiac malformations were present in 25/67 cases (37.3%). No chromosomal anomalies were present in fetuses with complete or corrected transposition of the great arteries. However, tetralogy of Fallot and double-outlet right ventricle were associated with chromosomal anomalies in 22% and 38% of cases, respectively, and with extracardiac anomalies in 45% and 46% of cases, respectively. Only 20 of the 67 (31%) cardiac malformations were associated with an abnormal four-chamber view. There were 28 (41.7%) terminations of pregnancy, six (8.9%) intrauterine deaths and 16 (23.8%) neonatal deaths. Seventeen neonates (25.3%) are currently alive, and 15 of these have undergone reparative surgery. The prognosis of conotruncal anomalies is poorer when the conditions is diagnosed in utero. This is mainly due to the frequent association with chromosomal and/or extracardiac anomalies, often leading to intrauterine or early neonatal death.
Electromechanical interaction, with prolonged QRS duration due to right ventricular (RV) overload, has been described as a predictor of unfavorable outcome in patients late after correction of Tetralogy of Fallot (TOF). Aim of our study was to evaluate myocardial function and activation delay of both left and right ventricles in TOF patients. Doppler echo, treadmill test and pulsed Tissue Doppler (TD) were performed in 25 healthy subjects and in 30 adult patients who had undergone surgery for TOF, all with right bundle branch block on ECG. Exclusion criteria were evidence of residual pulmonary either stenosis or regurgitation. By use of TD, the level of both LV mitral and RV tricuspid annulus were measured: systolic (Sm), early- and late-diastolic (Em and Am) regional peak velocities. The indexes of myocardial systolic activation were calculated: precontraction time (PCTm) and interventricular activation delay (InterV-del) (difference of PCTm between RV and LV segments). The two groups were comparable for LV diameters and for Doppler indexes, while QRS duration was prolonged and RV end-diastolic diameter was increased in TOF. By TD analysis, only at the level of tricuspid annulus TOF patients had lower Sm and Em, and increased RV PCTm ( p<0.001 ) and InterV-del ( p<0.0001 ), even after adjustment for heart rate (HR) and QRS duration. By treadmill test, TOF showed reduced cardiac functional reserve. In seven patients non-sustained ventricular tachycardia was documented during physical effort. By multivariate analysis, RV Em ( p<0.001 ), and InterV-del ( p<0.01 ) were independently associated to maximal workload at peak effort. The same InterV-del was an independent determinant of risk of ventricular arrhythmias during effort ( p<0.01 ). A cut-off point of Em peak velocity of tricuspid annulus <0.13 m/s at rest showed a sensitivity of 91% and a specificity of 88% in identifying TOF patients with submaximal exercise test. A cut-off point of InterV-del >55 ms showed 87% sensitivity and 88% specificity to detect increased risk of ventricular arrhythmias during effort. In TOF patients, TD analysis at rest may be taken into account as a non-invasive and easy-repeatable tool to predict cardiac performance during physical effort, and to select subgroups of patients at increased risk of ventricular arrhythmias.
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