Matteo Trezzi

Recurrent Aortic Prosthetic Valve Endocarditis: A Radical Additional Anatomical Solution

Recurrent prosthetic valve endocarditis, especially when characterized by annular abscess and aortic root destruction, remains a surgical challenge. A radical and aggressive surgical treatment is required preventing recurrent infection. Homograft implants are still thought to be the best surgical option, but they are not always available and their use in younger patient remains controversial. We propose an additional anatomical surgical technique, which consists in the implantation of a composite graft in the left ventricular outflow tract, well below the native aortic annulus, and then the direct reimplantation of coronary ostia.

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: primary repair

Primary repair of pulmonary atresia (PA) with ventricular septal defect (VSD) and major aortopulmonary collaterals based on single-stage unifocalization was first reported in 1995. From a midline approach, all collaterals are extensively dissected, translocated in front of the oesophagus and/or the trachea, when required, and directly anastomosed to each other or to the native pulmonary arteries, whenever present, without interposition of prosthetic material. The need for concomitant VSD closure is assessed intraoperatively with a pulmonary flow study according to a standardized protocol. Pulmonary blood supply is established by valved conduit interposition in all patients, regardless of the suitability for VSD closure. Palliation with systemic-pulmonary shunt is reserved for selected cases. Between 1994 and 2015, 94 patients with a median age of 1.09 years (range 0.03-19) underwent single-stage unifocalization at our institution. In 78 (82.1%) of them, an intraoperative pulmonary flow study was utilized to assess acceptability for concomitant VSD closure, which was accomplished in 69 cases (73%). Intraoperatively, following VSD closure, the mean right ventricle-to-aortic pressure ratio was 0.49 ± 0.14. The overall mortality rate was 11.2% (n = 10), with an 82% survival at 12.5 years. At a median follow-up interval of 5.8 years, the right ventricle-to-aortic pressure ratio did not differ significantly from the early postoperative phase. The surgical results of primary repair of PA with VSD and major aortopulmonary collaterals based on single-stage unifocalization and an intraoperative pulmonary flow study are satisfactory and durable, despite the need for repeated percutaneous or surgical reinterventions.

Congenital heart diseases and cardiovascular abnormalities in 22q11.2 deletion syndrome: From well-established knowledge to new frontiers

Congenital heart diseases (CHDs) and cardiovascular abnormalities are one of the pillars of clinical diagnosis of 22q11.2 deletion syndrome (22q11.2DS) and still represent the main cause of mortality in the affected children. In the past 30 years, much progress has been made in describing the anatomical patterns of CHD, in improving their diagnosis, medical treatment, and surgical procedures for these conditions, as well as in understanding the underlying genetic and developmental mechanisms. However, further studies are still needed to better determine the true prevalence of CHDs in 22q11.2DS, including data from prenatal studies and on the adult population, to further clarify the genetic mechanisms behind the high variability of phenotypic expression of 22q11.2DS, and to fully understand the mechanism responsible for the increased postoperative morbidity and for the premature death of these patients. Moreover, the increased life expectancy of persons with 22q11.2DS allowed the expansion of the adult population that poses new challenges for clinicians such as acquired cardiovascular problems and complexity related to multisystemic comorbidity. In this review, we provide a comprehensive review of the existing literature about 22q11.2DS in order to summarize the knowledge gained in the past years of clinical experience and research, as well as to identify the remaining gaps in comprehension of this syndrome and the possible future research directions.

Edwards Sapien 3 Valve for Mitral Replacement in a Child After Melody Valve Endocarditis

We present the surgical implantation in mitral position of the Edwards Sapien 3 valve for prosthetic valve endocarditis in a severely ill child after multiple valve replacements. The procedure was safely performed and provided adequate hemodynamic results in the short term. This report highlights the excellent potential of Sapien 3 valve for the treatment of mitral valve disease in children with a mitral valve annulus of appropriate size. Of note, the use of the Sapien 3 valve should be considered when a bioprosthesis is required at the time of valve re-replacement.

Long-Term Outcomes After Extracardiac Fontan Takedown to an Intermediate Palliative Circulation

BACKGROUND Acute failure of the Fontan circulation is rare but remains associated with high morbidity and mortality rates. Little is known about the long-term outcome of patients who underwent Fontan takedown to an intermediate palliative circulation and their potential candidacy for redo Fontan completion. METHODS Patients followed up at a single institution who underwent takedown of a Fontan circulation to an intermediate palliative circulation within 2 months of extracardiac Fontan completion were reviewed. RESULTS Between October 1990 and December 2015, 18 patients underwent Fontan takedown to a superior cavopulmonary connection (with or without an additional shunt) at a median age of 3.3 years (range, 1.8 to 8.0) and median weight of 13.8 kg (range, 8.0 to 27.0 kg). Takedown was required during the Fontan procedure itself in 2 patients, and within the first 2 postoperative months in 16 patients (median time to takedown, 3 days). Seventeen patients survived the post-takedown period and 3 ultimately underwent successful redo Fontan. Four patients required heart transplantation, with 2 deaths. In patients with extended intermediate palliation, median arterial oxygen saturation was 84% (range, 76% to 92%) at a median follow-up of 6.3 years (range, 0.7 to 25.9). CONCLUSIONS Takedown to a superior cavopulmonary connection is an effective treatment option and, in some patients, acts as a bridge to subsequent redo Fontan completion or heart transplantation. An extended intermediate palliative circulation is tolerated for several years with reasonable oxygen saturation levels at rest. In our experience, an early takedown strategy to a superior cavopulmonary connection is the treatment of choice for acute Fontan failure.

Intraoperative coronary revision but not coronary pattern is associated with mortality after arterial switch operation

OBJECTIVES We sought to determine differences in baseline characteristics and clinical outcomes in a consecutive series of patients undergoing arterial switch operation (ASO), assessing the effect of coronary anatomy on postoperative mortality, both overall and adjusted for surgical era. METHODS From January 2000 to May 2015, 283 consecutive patients underwent ASO for transposition of the great arteries. A total of 103 patients (36.4%) had an associated ventricular septal defect and 23 (8.1%) had an aortic arch obstruction. Coronary anomalies were present in 119 (42%) patients. RESULTS Overall survival was 93.0 ± 1% at 15 years (19 events). Intraoperative revision of the coronary anastomosis was required in 8 patients (2.8%) due to inability to wean from cardiopulmonary bypass (CPB) or post-CPB myocardial ischaemia. Surgical revision was neither related to the underlying coronary anatomy ( χ 2  =   0.681, P  = 0.4091) nor to the surgical era ( χ 2  =   0.682, P  = 0.4090). Univariable analysis suggested decreased mortality rate for infants who underwent surgery during 2008 or later ( P  = 0.01). In multivariable analysis, intraoperative coronary button revision was the only predictor of mortality [ P  < 0.001, hazard ratio (HR) 12.01, 95% confidence interval (CI) 3.85-37.49], whereas surgical era (before 2008) failed to achieve statistical significance ( P  = 0.057, HR 2.19, 95% CI 0.96-12.04). CONCLUSIONS In the recent era, unusual coronary patterns do not affect survival following ASO, whereas intraoperative coronary button revision is a predictor of mortality. Preventive strategies aimed towards intraoperative detection of impaired coronary perfusion are useful tools that might be considered for all patients in order to improve outcomes.

Pulmonary Arteries: Surgical Point of View

Surgical repair of the pulmonary artery (PA) branches encompasses many different clinical scenarios and technical challenges. Branch PA stenosis can occur either primarily or following a previous attempt at surgical repair. In cases of complex congenital heart disease (CHD) requiring reoperations or staged palliation, surgical patch angioplasty is often needed and may prove challenging. Aside from elective indications, surgical PA plasty is necessary following complications of percutaneous stent implantation such as PA dissection or rupture, stent migration, suboptimal stent expansion, stent thrombosis, stent restenosis, airway compression, and inhibition of PA growth. The main challenge in PA surgery is to obtain a spatially pleasing reconstruction in three dimensions.