Nicola Maschietto

Pulmonary hypertension in sickle cell disease children under 10 years of age.

Despite the finding of elevated Tricuspid Regurgitant Velocity (TRV) in children below 5 years of age, the prevalence and evolution of Pulmonary Hypertension (PH) in young children with sickle cell disease (SCD) are unclear. In order to identify predictive factors of precocious PH development, SCD children ≥3 years old, at steady state, underwent annual echocardiography and Tissue Doppler Imaging (TDI). Patients receiving chronic transfusion were excluded. Thirty‐seven of seventy‐five patients were ≥3 years, with measurable TRV. In our young population (mean age 6·2 years) of mainly African, HbS/HbS patients, 8/37 (21·6%) had TRV ≥2·5 m/s, 8% being only 3 years old. Significant correlation was found between precocious TRV elevation and high platelet and reticulocyte counts and frequent acute chest syndromes (ACS). In multivariate analysis, ACS was the only variable predicting TRV ≥2·5 m/s. TDI of the 37 patients showed signs of diastolic dysfunction of the left ventricle. At follow‐up all eight patients with high TRV displayed further increase and seven more developed TRV ≥2·5 m/s. PH seems to begin in children earlier than expected. Factors involved in its early onset might be different from the ones causing its development in older children or adults. African children might benefit from early screening and re‐assessment once a year.

The balloon dilation of the pulmonary valve during early repair of tetralogy of Fallot

Progressive pulmonary valve (PV) regurgitation leads to right ventricular failure after repair of tetralogy of Fallot (TOF). We sought to evaluate our results with the preservation of the PV in selected patients with TOF.

Anomalous origin of one pulmonary artery from the ascending aorta

UNLABELLED We describe the surgical repair in three infants presenting with one pulmonary artery arising from the ascending aorta, the other artery arising normally from the right ventricle via the pulmonary trunk. Repair consisted of reimplantation of the anomalous pulmonary artery to the pulmonary trunk, in association with repair of associated intracardiac malformations. All patients survived the surgical procedures, and were discharged in stable clinical condition. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and underwent reoperation. Although survival after operation is now expected for this malformation, reports of late results are lacking. Larger numbers of operations are needed before we can reach definitive conclusions. The origin of one branch pulmonary artery from the ascending aorta in the presence of a pulmonary valve and main pulmonary artery is a very rare congenital cardiac anomaly. PATIENTS AND METHODS Between January 1995 and June 2003, 3 infant girls presented with the origin of one branch artery from the ascending aorta, while the other pulmonary artery originated from the pulmonary trunk which was in continuity with the right ventricular outflow tract. The pulmonary artery that arose from the right ventricle was left in 2 and right in 1 patient. RESULTS At the age 13, 48 and 62 days respectively, the patients underwent surgical repair consisting with reimplantation of the anomalous pulmonary artery branch to the pulmonary trunk in association with repair of intracardiac malformations. There were no hospital deaths. Postoperative complications included: prolonged intubation in two patients (10 and 16 days), low output syndrome in 1 patient, cardiac tamponade in 1 patient and seizures in 1 patient. All patients were discharged in good clinical condition. There have been no late deaths. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and after unsuccessful balloon dilation, underwent surgical reoperation. CONCLUSIONS Although operative survival is now possible for this malformation, reports of late results are lacking. Two of the three patients developed stenosis at the surgical anastomosis relatively early after surgery. Larger numbers of operations are necessaries to reach definitive conclusions.

Cardiac Operations After Patent Ductus Arteriosus Stenting in Duct-Dependent Pulmonary Circulation

BACKGROUND Stenting of the patent ductus arteriosus (PDA) has been recently introduced to palliate patients with duct-dependent pulmonary circulations. We evaluated the surgical outcome of patients who had a previous PDA stent, focusing on their pulmonary arteries status. METHODS This study included 15 patients (11 boys, 4 girls) who underwent cardiac operations after PDA stenting between August 2004 and April 2009. Outcomes included hospital mortality, morbidity, and need for reintervention or operation on the PDA and on the pulmonary artery branches. RESULTS Thirteen patients underwent elective cardiac operations at a median of 11 months (range, 0.3 months to 3.7 years) from PDA stenting. Two patients underwent emergency operations due to stent migration during percutaneous positioning. Six patients (46%) required a preoperative interventional cardiology procedure, including PDA stent dilatation in 5 and multiple left pulmonary artery dilatations in 1. During elective surgical repair, PDA stents were completely retrieved in 3 patients (23%) and partially removed in 10 (77%) due to the fusion of the stent to the vascular wall. Seven patients (53.8%) required surgical pulmonary artery plasty. One in-hospital death (6%) occurred after an emergency operation. Median follow-up was 16.7 months (range, 1 month to 2.5 years). Two late deaths (14%) occurred at 4 and 9 months. Four patients required additional interventional procedures on the left pulmonary artery after surgical repair. CONCLUSIONS Operations after PDA stenting are safe and low-risk. The presence of PDA stents requires additional surgical maneuvers on pulmonary arteries in near half of the patients, and postoperative interventions can be required.

Surgical outcomes of total anomalous pulmonary venous connection repair: a 22-year experience.

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease (CHD), whose surgical repair is associated with high mortality and reoperation rates. We sought to identify predictors of early and late outcomes.

Two-dimensional, M-mode and Doppler-derived echocardiographic parameters in sedated healthy growing female sheep:

Despite the fact that sheep are a widely used animal model in cardiovascular research, reference values for transthoracic echocardiography in normal growing animals are not available. Eight healthy female lambs underwent two-dimensional, M-mode and pulsed wave Doppler echocardiographic examination at 100 days of age and every three months thereafter over a 12-month period. The study was conducted under sedation with midazolam, butorphanol and constant rate infusion of intravenous propofol. Their growth phase was completed at about one year of age. All the echocardiographic parameters considered were significantly correlated with body weight and age class except for the left ventricular systolic and diastolic diameters. Functional indices were not correlated to body weight or age except for the E-point to septal separation distance (EPSS). Doppler-derived parameters were not influenced by independent variables. Transthoracic echocardiography can be considered an applicable method for cardiovascular research using a growing lamb animal model after appropriate adjustments for age and body size.

Novel valve replacement with an extracellular matrix scaffold in an infant with single ventricle physiology

Valve replacement in children with functionally univentricular hearts remains challenging. The absence of small prostheses, the lack of growth, and the need for anticoagulation limit these procedures. We describe a 1-year follow-up of an extracellular matrix scaffold tube used as systemic atrio-ventricular valve in an infant.

Two-dimensional and real-time three-dimensional echocardiographic fetal diagnosis of aorto-ventricular tunnel

A 22-gestational-week-old female fetus was referred to our prenatal echocardiographic unit to evaluate her left ventricular dilatation and dysfunction. An aorto-ventricular tunnel was visualized (Figure 1a and 1b), causing marked left ventricular diastolic overload, overt fetal heart failure, hydrops, and polidramnios. The mother was informed about the possibility of spontaneous abortion or premature delivery, and the pregnancy was closely monitored with serial fetal echographic and echocardiographic evaluations. At 33 weeks of gestational age (GA), the signs of fetal heart decompensation were still present, but the fetus’ vitality and growth …

Congenital heart disease in adults: an 8-year surgical experience in a medium-volume cardiac center.

Objectives We report our 8-year experience with surgery for congenital heart disease in adults in a medium-volume surgical center. Methods We collected data of 161 consecutive patients who underwent 230 procedures from January 1997 to December 2004. Surgical procedures were divided into two groups: repair (85.7%) and reoperation (14.3). Results There was only one in-hospital death (Fontan revision, in reoperation group). At a mean follow-up time of 56 ± 30 months, overall freedom from any kind of complication is 76.5%. Cox analysis showed that incremental risk factors were preoperative cyanosis, reoperation, and length of ICU stay (hazard ratio = 4.47, 3.34 and 1.49, respectively; P < 0.001 in all), whereas decremental risk factors were preoperative New York Heart Association class 1 (hazard ratio = 0.328, P < 0.001) and surgery for ‘septal defect’ (hazard ratio = 0.26, P = 0.02). Conclusion Surgery for congenital heart disease in adults seems to be an overall well-tolerated and a low-risk treatment, with overall good mid-term clinical results.

One-and-a-Half Ventricle Repair as a Surgical Alternative to Fontan Revision in an Adult

We report a case of a young man with hypoplastic right ventricle, who presented with recurrent untreatable arrhythmias after a Bjork Fontan procedure in infancy. He underwent one‐and‐a‐half ventricle repair as an alternative surgery to Fontan revision. doi: 10.1111/jocs.12410 (J Card Surg 2014;29:832–835)