Lucia Rossetti

Usefulness of Bicuspid Homograft Reconstruction of the Right Ventricular Outflow Tract in Infants With Complex Congenital Heart Disease

Homograft supply is limited especially for those sizes required for neonatal and infant cardiac operations. Surgically downsized homografts have been successfully used for right ventricular outflow tract reconstruction in 5 children (mean weight 6.2 kg) with evidence of good hemodynamic performance of the conduits at a mean follow-up time of 3.7 years.

Long-Term Follow-Up Study of Temporary Tricuspid Valve Detachment as Approach to VSD Repair without Consequent Tricuspid Dysfunction

Temporary tricuspid valve detachment improves the operative view of certain congenital ventricular septal defects (VSDs), but its long-term effects on tricuspid valve function are still debated. From 2002 through 2012, we performed a prospective study of 68 children (mean age, 1.28 ± 1.01 yr) who underwent transatrial closure of VSDs following temporary tricuspid valve detachment. Sixty patients had conoventricular and 8 had mid-muscular VSDs. All were in sinus rhythm. Seventeen patients had systemic pulmonary artery pressures. Preoperative echocardiograms showed trivial-to-mild tricuspid regurgitation in 62 patients and tricuspid dysplasia with severe regurgitation in 6 patients. Patients were clinically and echocardiographically monitored at 30 postoperative days, 3 months, 6 months, every 6 months thereafter for the first 2 years, and then once a year. No in-hospital or late death was observed at the median follow-up evaluation of 5.9 years. Mean intensive care unit and hospital stays were 1.6 ± 1.1 and 7.3 ± 2.7 days, respectively. Residual small VSDs occurred in 3 patients, and temporary atrioventricular block in one. After VSD repair, 62 patients (91%) had trivial or mild tricuspid regurgitation, and 6 moderate. Five of these last had severe tricuspid regurgitation preoperatively and had undergone additional tricuspid valve repair during the procedure. The grade of residual tricuspid regurgitation remained stable postoperatively, and no tricuspid stenosis was documented. All patients were in New York Heart Association class I at follow-up. Temporary tricuspid valve detachment is a simple and useful method for a complete visualization of certain VSDs without incurring substantial tricuspid dysfunction.

Ischemia Induced by Transesophageal Atrial Pacing Stress Echocardiography Predicts Long-Term Mortality

Objectives: It was the aim of this study to investigate the long-term value of transesophageal atrial pacing in predicting death in patients with known or suspected coronary artery disease. Background: Exercise, dobutamine and dipyridamole stress echocardiography are all effective in predicting cardiac death. Transesophageal atrial pacing stress echocardiography (TAPSE) is a safe alternative to pharmacologic tests, but no information is available on prognosis with TAPSE. Methods: One thousand and ten TAPSE were performed in 975 consecutive patients. TAPSE was feasible in 970 tests (96%); after exclusion of the 35 patients with more than 1 TAPSE and those 42 lost at follow-up (mean 4.5 ± 3.7 years, median 6 years), the final population consisted of 857 patients (675 males, 58 ± 9 years old). The Cox model was used to analyze the association of clinical, resting and TAPSE variables with cardiac death. Results: TAPSE was abnormal in 281 (32%) patients. There were 46 cardiac-related deaths (5%), 25 among the 281 patients with an abnormal test (8.9%) and 21 among the 576 patients with a normal test (3.6%). The predictors of cardiac death were age, previous revascularization, resting wall motion score index and its variation during TAPSE. Abnormal TAPSE significantly increases the value of models predicting cardiac death. Moreover, cardiac mortality increased progressively with the extent of the induced ischemia. Conclusions: TAPSE is a useful tool in predicting death in patients with known or suspected coronary artery disease and might be considered an alternative to pharmacologic stressors.

Current management of double-outlet left ventricle: towards biventricular repair in infancy.

Background Double-outlet left ventricle (DOLV) is an extremely rare type of ventriculoarterial connection, mostly reported anecdotally with wide variations in morphology, surgical strategy and clinical outcome. The aim of the present study was to define current morbidity and mortality of biventricular repair and discuss surgical management in infancy. Methods Inclusion criteria were English literature publication; balanced ventricular development and completion of biventricular repair. Institutional experience was also reported. Study end points were survival and freedom from cardiovascular events. Univariate analysis to identify risk factors for complications was performed. Results During a 45-year interval, 37 cases of DOLV were identified. Median age at repair was 48 months (range 0.3–336); seven patients were infants. Extracardiac repair was performed in 21 (57%) patients, right ventricular outflow patch in seven, pulmonary translocation in six, and intraventricular repair in three. There were three (8%) hospital deaths, due to right heart failure, and seven (21%) patients experienced 10 perioperative complications. Univariate analysis showed year of operation (P = 0.02) and outflow patch technique (P = 0.003) as risk factors for hospital morbidity and mortality. During a median follow-up of 26 months (range 1–192), there was one death and seven reoperations. Ten-year survival and freedom from adverse cardiovascular events were 87 ± 6 and 70 ± 10%, respectively. Conclusion Although seldom reported in the past, repair of DOLV in infancy does not increase surgical risk. Early and late morbidity are mostly due to right heart complications and more common after right ventricular outflow patch repair. Paucity of follow-up information may underestimate actual late adverse event rate.

Twenty‐Year Outcome After Right Ventricular Outflow Tract Repair Using Heterotopic Pulmonary Conduits in Infants and Children

Durability of pulmonary conduits (PCs) used for reconstruction of the right ventricular outflow tract (RVOT) may be affected by a variety of factors. Among these, the technique used for PC implantation, whether in orthotopic or heterotopic position, strictly dependent upon the underlying anatomy, has been suggested to influence long-term outcome after RVOT repair. To determine the outcome of heterotopic implantation in infants and children treated at our institution, late results of heterotopic PC in non-Ross patients were analyzed and compared with data of orthotopic PC in age-matched pediatric Ross patients operated during the same time period. Between November 1991 and January 2015, 58 infants and children, 32 male and 26 female, with a median age of 9.4 years (range 1 day-18 years) underwent implantation of heterotopic PC (31 homografts [HG] and 27 xenografts [XG]) for reconstruction of RVOT. Median age in the XG group was significantly lower than in the HG group (0.9 vs. 13.4 years, P = 0.01), while male/female ratio was similar. Fifty (86%) patients had undergone one or more prior cardiac operations, while 32 (55%) required associated procedures during PC implantation. Comparison with data in 305 children and with a median age of 9.4 years, receiving orthotopic PC between 1990 and 2012 (Italian Pediatric Ross Registry), was undertaken. Descriptive, univariate, and Kaplan-Meier analysis defined outcome. There were three (5.2%) early and five (9.0%) late deaths, during a median follow-up of 7.6 years (range 2 months-23 years). Patients having XG had trend toward higher hospital mortality (2/27 vs. 1/31, P = 0.2), but similar late mortality (2/24 vs. 3/30, P = 0.3). Overall survival was 88 and 62%, while freedom from PC replacement was 49 and 21%, at 10 and 20 years, respectively. The latter proved significantly worse than freedom from orthotopic PC replacement, which was 94 ± 2 and 70 ± 9% at 10 and 20 years (P = 0.02). When stratified for type of heterotopic PC, late survival proved comparable (81 and 81% for XG vs. 92 and 60% for HG, at 10 and 20 years, respectively, P = 0.7). However, freedom from PC replacement was significantly higher in patients with heterotopic HG (21 and 5% for XG vs. 63 and 48% for HG, at 10 and 20 years, respectively, P = 0.001). RVOT repair using either XG or HG in heterotopic position is a safe procedure associated with low hospital mortality and satisfactory late survival. Freedom from reoperation is significantly lower than that observed in age-matched children having orthotopic HG. Freedom from reoperation in heterotopic XG is poorer than in HG, although different baseline demography may have influenced this finding.

Clinical Outcome of Benign Cardiac Tumors inInfants During A 13 Years’ Experience: Impact ofPrenatal Diagnosis

Background: Primary cardiac tumors (PCT) in children are rare disorders. Prenatal diagnosis is feasible. Most of them have favorable prognosis, but some still prove fatal.Objective: To define clinical outcome of benign PCT and the possible impact of prenatal diagnosis. Methods: 14 cases were collected between December 2005 and August 201. We included patients with pre and postnatal diagnosis, medically and surgically managed. Pre and postnatal examinations, surgical procedures, clinical information before and after surgery, histological diagnoses were reviewed. Results: Seventy-two percent of PCT were detected prenatally. Median follow-up time was 3,13 years. In the medical managed group, one had complete spontaneous regression, 3 had spontaneous reduction, 2 underwent efficacious Enviroximes treatment and one was lost to follow-up. 46% of cases needed surgery. In two infants, perioperative support with V-A ECMO and iNO was necessary. Complete excision of the mass was possible in 5 cases. Rate of hospital death was 20%. All but one late survivors were symptom-free at last clinical assessment. Clinical relevant recurrence of mixoma occurred in one case. Conclusion: Benign PCT have extremely diverse course, some proving fatal in spite of benign histology. Prenatal diagnosis is crucial to plan delivery and neonatal management, as some benign PCT require intensive care. Complete excision of the mass usually is feasible but may be limited by young age and extent of cardiac infiltration. Even if patients are generally well after surgery, long term follow up is mandatory as some masses recur.

Two Cases of Double-Outlet Left Ventricle Detected Prenatally

Double-outlet left ventricle is an exceedingly rare congenital heart defect. Its prenatal detection and precise anatomical definition are challenging for a variety of reasons and have never been previously reported. Here described are 2 cases of prenatally diagnosed double-outlet left ventricle. The technical limitations of prenatal diagnosis and its implications for the surgical management of patients affected by such a rare condition are discussed.