Pregnancy after surgical correction of tetralogy of Fallot.

Forty pregnancies have been documented in 27 patients with surgically corrected tetralogy of Fallot. Infertility was uncommon and there were no premature births and few abortions or small-for-dates babies; this suggests that surgery that corrects cyanosis improves the outcome of pregnancy by correcting the fetal environment. Pregnancy was well tolerated and there were no serious cardiac complications. Thirty of the 31 infants examined were normal, the one abnormal infant having pulmonary atresia. A patient with no major residual defects after surgery for tetralogy of Fallot may be reassured that pregnancy will be well tolerated and that delivery may be managed in the normal manner.     

Arrhythmias after tetralogy of fallot repair

Tetralogy of Fallot is the most common cyanotic congenital heart disease, with a good outcome after total surgical correction. In spite of a low perioperative mortality and a good quality of life, late sudden death remains a significant clinical problem, mainly related to episodes of sustained ventricular tachycardia and ventricular fibrillation. Fibro-fatty substitution around infundibular resection, intraventricular septal scar, and patchy myocardial fibrosis, may provide anatomical substrates of abnormal depolarization and repolarization causing reentrant ventricular arrhythmias. Several non-invasive indices based on classical examination such as ECG, signal-averaging ECG, and echocardiography have been proposed to identify patients at high risk of sudden death, with hopeful results. In the last years other more sophisticated invasive and non-invasive tools, such as heart rate variability, electroanatomic mapping and cardiac magnetic resonance added a relevant contribution to risk stratification. Even if each method per se is affected by some limitations, a comprehensive multifactorial clinical and investigative examination can provide an accurate risk evaluation for every patient.     

Long-term follow-up after relapses in children with Hodgkin's disease

In the years 1969-1980, 68 children with Hodgkin's disease were subjected to a combined MVPP and radiotherapy. Remissions were obtained in 64 patients, and relapses occurred in 11 children. The treatment of relapse consisted in administration of B-DOPA alone or alternatively with MVPP combined with radiotherapy (in 7 out of 11 patients). The patients were recycled every 2-3 weeks which, with other modifications of chemotherapy, allowed for the completion of the six first cycles within the period of 4,5 to 7 months. A relapse caused death of one child, and two others demonstrated further relapses. At present eight children have been showing disease-free survival following a relapse for the period of 29+ to 152+ (median, 94.5 months). The authors concluded that in patients with relapses of Hodgkin's disease the decisive role rests upon aggressive chemotherapy (high frequency of cycles).     

Numerical investigation of regurgitation phenomena in pulmonary arteries of Tetralogy of Fallot patients after repair

Pulmonary regurgitation is a very common phenomenon in pulmonary arteries after repair of patients of Tetralogy of Fallot (TOF) which is the most common complex congenital heart diseases. The aim of this study is to use numerical approaches to simulate flow variations in pulmonary artery after repair of patients of TOF. We analyze the flow patterns in an in-vitro bifurcation pulmonary artery and consider effects of various regurgitation fractions (RF or b/f) in left pulmonary artery (LPA) and right pulmonary artery (RPA). We not only observe the variation of flow patterns, but also analyze the results of b/f and net volumetric flow rates in LPA and RPA. In general, the b/f of LPA is higher than RPA in the measured data provided by phase-contrast magnetic resonance imaging (PC-MRI). We validate the result using numerical approaches to analyze the flow patterns in pulmonary artery in this study. The results will be useful for medical doctors when they perform operations for TOF patients.     

22q11 deletions in isolated and syndromic patients with tetralogy of Fallot

Tetralogy of Fallot (TF) is a congenital conotruncal heart defect commonly found in DiGeorge (DGS) and velo-cardio-facial (VCFS) syndromes. The deletion of chromosome 22q11 (de122q11) is a well established cause of DGS and VCFS, and it has been demonstrated also in sporadic or familial cases of TF. In order to investigate the prevalence of de122q11 in patients with TF, we analyzed the DNA of 137 consecutive patients with syndromic and isolated TF, using the HD7k probe, which detects hemizygosity for the D22S134 locus. De122q11 has been detected in 11/26 (42%) syndromic patients. Evidence for hemizygosity was obtained in all patients with DGS and in 8/15 patients with VCFS. None of the 107 patients with isolated TF had de122q11. Our experience suggests that children with TF and de122q11 always present major or minor extracardiac anomalies. These features, including subtle facial dysmorphisms, should be checked routinely in patients with TF and other conotruncal heart defects.     

Long-term follow-up studies after homograft replacement of the mitral valve

Follow-up studies on 132 patients who have received fresh aortic homograft replacement of the mitral valve since May 1967 indicate good long-term function of the valve. Clinically the majority of patients are greatly improved and are free from the risks of long-term anticoagulant therapy. Hemodynamic studies performed on 13 patients at 25 to 41 months postoperatively showed a significant decrease in left atrial and pulmonary artery pressures with a small increase in cardiac output. Late deterioration of the homograft produced severe insufficiency in four cases and organic stenosis in two cases. Reasons for isolated deterioration are suggested.     

Ten-year follow-up after endovascular repair of traumatic abdominal aortic rupture

Blunt abdominal aortic injury is often associated with bowel injury that precludes operative repair because of the risk of graft infection. Endovascular repair has been reported but with limited follow-up. We present a case of a 15-year-old boy who underwent endovascular repair of blunt abdominal aortic rupture and whom we were able to follow up over a decade. Our experience with this case and three others, as well as the experience reported in the literature, suggests that endovascular repair is a reasonable option in the setting of concomitant bowel injury. The risk of over sizing, collapse, and migration may be less than that described for thoracic aortic injuries because there is no need to deploy the endograft across an angle.