The Appraisal of Adults with Congenital Heart Disease: Lesson from Comparison of Surgical Outcomes.

The population of adults with congenital heart disease (ACHD) is constantly growing. There seems to be a consensus that these patients are difficult to manage especially if compared to patients with acquired heart disease. The aim of this study is to compare outcomes and results of cardiac surgery in ACHD patients with a reference population of adults with acquired cardiac disease. Retrospective study of 5053 consecutive patients older than 18 years hospitalized for cardiac surgery during a 5-years period in our Institution. Two groups of patients were identified. Group I: 419 patients operated for congenital heart disease; Group II: 4634 patients operated for acquired heart disease. In each Group were identified low, medium, and high-risk patients, according to validated scores. Right ventricular outflow tract surgery was the most frequent procedure in Group I, while coronary artery by-pass grafting was the most common in Group II. Patients with ACHD were younger (37.8 vs. 67.7 years), with higher number of previous operations (32.1% vs. 6.9%), had longer post-ICU hospital stay (11 vs. 8 days) but had lower ICU stay (1 vs. 2 days), shorter assisted mechanical ventilation (12 vs. 14 h) and lower surgical mortality (1 vs. 3.7%) (all p < 0.001). No differences were found in term of post-operative complications (12.4 vs. 15%). The surgical treatment of ACHD patients can be done with excellent results and if compared with acquired cardiac disease patients they have better results with shorter ICU stay and lower mortality.

T-Remodeling of the Pulmonary Artery Bifurcation for Pulmonary Artery Origin Stenosis.

BACKGROUND: Various surgical techniques are utilized for reconstructing hypoplastic pulmonary arteries (PAs) in patients with conotruncal anomalies and at times, may be susceptible to restenosis and reoperation. We reviewed our experience with a simple technique of T-shaped remodeling of the PA bifurcation. METHODS: Between 2005 and 2019, 31 patients underwent T-remodeling of central PAs by a single cardiac surgeon. The PA bifurcation was opened cranially, and the opening was augmented with an oval-shaped patch effectively transforming the V-shaped bifurcation into a T-shaped bifurcation. Both origins of the PAs were enlarged, even in the instance of single PA origin stenosis. RESULTS: Median age at time of T-remodeling was 17 months (range: 7 weeks to 14 years). The following cardiac morphologies were observed: tetralogy of Fallot (n = 12, 39%), pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (n = 8, 26%), truncus arteriosus (n = 6, 19%), pulmonary atresia with VSD (n = 3, 9.7%), and transposition of the great arteries (n = 2, 6.5%). Thirteen patients (42%) had previous central shunt, and eight patients (26%) had previous modified Blalock-Taussig shunt. There were no operative mortalities. Immediately after T-remodeling, echocardiographic estimates of right ventricle to PA gradient decreased from 42 [interquartile range 28-58] mm Hg to 20 [12-36] mm Hg (P = .03). Freedom from reoperation on the PA bifurcation for the entire cohort was 100% at one year, 88% (95% CI 68%-96%) at five years and 82% (57%-93%) at ten years. CONCLUSIONS: T-remodeling for PA origin stenosis is a safe procedure with excellent freedom from reoperation that is easily reproducible and applicable to patients with all cardiac morphologies.

Impact of COVID-19 Pandemic on the Italian Humanitarian Congenital Cardiac Surgery Activity: What No One Tells You.

More than 4 millions of children with congenital heart disease (CHD) are waiting for cardiac surgery around the world. Few of these patients are treated only thanks to the support of many non-governmental organizations (NGOs). Starting in December 2019, the so-called coronavirus disease 2019 (COVID-19) has rapidly become a worldwide pandemic and has dramatically impacted on all the international humanitarian activities for congenital heart disease. We analyzed data from all the Italian congenital cardiac surgery centers with the aim to quantify the impact of the pandemic on their charities. Fifteen Italian centers participated in the study and contributed to data collection. We analyzed and compared data regarding humanitarian activities carried out abroad and on site from two periods: year 2019 (pre-COVID-19) and year 2020 (COVID-19 pandemic). In 2019, 53 international missions were carried out by Italian congenital cardiac surgeons, resulting in the treatment of 471 CHD patients. In the same period 11 Italian cardiac centers operated on 251 foreign patients in Italy. In 2020, the pandemic led to a reduction of this activity by 96% for the surgery performed overseas and 86% for the interventions carried out in Italy. In conclusion our study shows the important quantitative impact of the pandemic on the Italian humanitarian cardiac surgical activity overseas and in Italy. This shocking result highlights the failure of the systems adopted so far to solve the problem of CHD in developing countries.

Replacement of the Mitral Valve Under One Year of Age: Size Matters.

Surgical management of mitral valve disease in neonates and infants is challenging. When repair is no longer feasible, replacement may become inevitable, but should only be considered as an option of last resort due to the remarkably high rate of associated morbidity and mortality. Mechanical valves are the preferred choice in large annuli, while stented conduits seem promising in smaller ones. In patients with a preoperative mitral valve annulus equal or larger than 15-16 mm, an intra-annular placement of the smallest mechanical valve available should be attempted. In patients with smaller annuli, the placement of a stented valved conduit seems to display a lower mortality risk. Supra-annular implantation of prostheses should be reserved for exceptional cases and to those familiar with this technique because of the high rate of associated complications.

Surgical Rescue After Failed Percutaneous Closure of an Aorto-Atrial Tunnel.

Congenital sinus of Valsalva aneurysm is a rare disorder, mostly involving the right and noncoronary sinuses, in which intracardiac rupture is more likely foreseen into the right chambers. Because of the unfavorable prognosis, which includes heart failure and sudden death, detection itself is an indication for treatment, which may be performed either surgically or percutaneously. We present a case of a four-year-old patient with aorto-right atrial tunnel, in which a transcatheter attempt of closure was performed, complicated by new onset of aortic valve regurgitation, requiring surgical intervention.

Outcomes and Quality of Life After Ross Reintervention: Would You Make the Same Choice Again?

BACKGROUND: The Ross procedure was introduced as a long-term if not definitive solution for aortic pathology. However, the rate of reoperation is not negligible. METHODS: This single-center prospective study assessed the general outcome of Ross reoperation and patients' perceived quality of life compared with 2 control groups (Ross non-reoperation and mechanical aortic valve replacement). Patient's preference regarding the choice between mechanical aortic valve and Ross procedure was investigated in a subgroup that could theoretically have been directed to either of the 2 procedures. RESULTS: Between 2005 and 2017, 64 consecutive patients underwent reoperation after Ross. Median age was 31 years. Median freedom from reoperation after the Ross procedure was 136 months. An autograft reoperation was required in 49, and 25 had homograft failure. No in-hospital death was recorded. Mean follow-up was 77 months (range, 6-164 months). Quality of life was assessed with the 36-Item Short Form Health Survey questionnaire. The Ross reoperation group showed a lower score involving psychological concerns compared with the other groups. In the reoperated-on patients group, 52 had adequate aortic annulus dimensions to receive a prosthetic valve instead of a Ross procedure. When asked whether they would make the same choice, only 31% confirmed the preference. CONCLUSIONS: Reoperations after Ross procedure have low mortality and morbidity. Long-term follow-up showed a high quality of life, even after reoperations. However, owing to psychological concerns after the redo operation, when choosing a Ross procedure, it is our duty to thoroughly explain to patients that a high level of disillusion is predictable in case of reoperations.

Perfusion Study Helps in the Management of the Intraseptal Course of an Anomalous Coronary Artery.

Anomalous origin of a coronary artery from the opposite aortic sinus of Valsalva can present in various ways, ranging from a benign and incidental finding to sudden cardiac death. The variant with an intraseptal subpulmonary course (sometimes referred to as intraconal), is widely perceived to carry a low risk of ischemia and has been considered to be a benign variant, not requiring surgical treatment. In one of our recent patients, however, nuclear scintigraphy highlighted a myocardial perfusion deficit in the territory supplied by the allegedly benign anomalous coronary artery, prompting the need for a more aggressive surgical approach.

Anomalous aortic origin of coronary arteries: Early results on clinical management from an international multicenter study.

BACKGROUND: Anomalous aortic origin of coronary arteries (AAOCA) is a rare abnormality, whose optimal management is still undefined. We describe early outcomes in patients treated with different management strategies. METHODS: This is a retrospective clinical multicenter study including patients with AAOCA, undergoing or not surgical treatment. Patients with isolated high coronary take off and associated major congenital heart disease were excluded. Preoperative, intraoperative, anatomical and postoperative data were retrieved from a common database. RESULTS: Among 217 patients, 156 underwent Surgical repair (median age 39 years, IQR: 15-53), while 61 were Medical (median age 15 years, IQR: 8-52), in whom AAOCA was incidentally diagnosed during screening or clinical evaluations. Surgical patients were more often symptomatic when compared to medical ones (87.2% vs 44.3%, p < 0.001). Coronary unroofing was the most frequent procedure (56.4%). Operative mortality was 1.3% (2 patients with preoperative severe heart failure). At a median follow up of 18 months (range 0.1-23 years), 89.9% of survivors are in NYHA ≤ II, while only 3 elderly surgical patients died late. Return to sport activity was significantly higher in Surgical patients (48.1% vs 18.2%, p < 0.001). CONCLUSIONS: Surgery for AAOCA is safe and with low morbidity. When compared to Medical patients, who remain on exercise restriction and medical therapy, surgical patients have a benefit in terms of symptoms and return to normal life. Since the long term-risk of sudden cardiac death is still unknown, we currently recommend accurate long term surveillance in all patients with AAOCA.

Surgical ablation of ventricular tachycardia in patients with repaired tetralogy of Fallot†.

OBJECTIVES: Life expectancy of patients with repaired tetralogy of Fallot is normal until adulthood, when it becomes burdened by major complications such as cardiac arrhythmias and heart failure. Ventricular tachycardia (VT), associated with electrical conduction abnormalities in anatomical isthmuses, is the most common complication and is refractory to medical therapy. The arrhythmogenic sources can be surgically ablated during reoperations for pulmonary valve incompetence. The purpose of our study is to evaluate the safety and effectiveness of surgical radiofrequency ablation during PVR and its long-term impact on the occurrence of cardiac events. METHODS: A total of 118 patients with repaired tetralogy of Fallot underwent reoperation for pulmonary valve regurgitation in IRCCS-Policlinico San Donato between January 2002 and February 2016. Twenty patients with risk factors for ventricular arrhythmia underwent EP testing and were found positive. The mean QRS duration was 178 ms. Right ventricular dysfunction was present in all the patients, and 20% had associated tricuspid regurgitation. The cohort (N = 20), with positive EP study, underwent pulmonary valve replacement and concomitant intraoperative ventricular surgical radiofrequency ablation, targeting the slow conducting isthmuses identified during preoperative electroanatomical mapping. All patients were reviewed at 1 month, 3 months and 6 months with clinical evaluation, echocardiography, ECG and implantable cardioverter-defibrillator interrogation. At 6 months, a postoperative electrophysiological study was performed in all patients, and a cardioverter defibrillator was implanted as primary preventive measure in patients with residual inducible VT. RESULTS: Pulmonary valve replacement and surgical ventricular radiofrequency ablation were performed in all cases. One patient died while on V-V extracorporeal membrane oxygenation (ECMO) due to respiratory failure. There was no late mortality. Follow-up was complete with a median of 6.5 years. During the postoperative EPS study, inducible VT was found in 15.7% of patients who received an implantable cardioverter-defibrillator, whereas VT could no longer be induced in the remaining patients. No procedure-related complication was observed, and freedom from ventricular arrhythmia was 94% and 89.5% at 1 year and 5 years, respectively. CONCLUSIONS: Pulmonary regurgitation and right ventricular dysfunction correlate with VT and SCD, but valve replacement alone does not reduce the risk of ventricular tachyarrhythmias. Intraoperative ventricular ablation during reoperation for pulmonary regurgitation prevents the occurrence of ventricular arrhythmias in the early and medium term. Accordingly, this technique may be proposed as a safe associated procedure in selected patients.

Subclavicular arteriovenous fistula for univentricular heart palliation.

Creation of a subclavicular arteriovenous fistula is a minimally invasive procedure that provides an additional source of arterial blood flow to the pulmonary arteries in patients who have a Glenn shunt but are unsuitable for or at high risk for Fontan completion.

Venoarterial Extracorporeal Membrane Oxygenation for Acute Fulminant Myocarditis in Adult Patients: A 5-Year Multi-Institutional Experience.

BACKGROUND: Acute fulminant myocarditis (AFM) may represent a life-threatening event, characterized by rapidly progressive cardiac compromise that ultimately leads to refractory cardiogenic shock or cardiac arrest. Venoarterial extracorporeal membrane oxygenation (VA-ECMO) provides effective cardiocirculatory support in this circumstance, but few clinical series are available about early and long-term results. Data from a multicenter study group are reported which analyzed subjects affected by AFM and treated with VA-ECMO during a 5-year period. METHOD: From hospital databases, 57 patients with diagnoses of AFM treated with VA-ECMO in the past 5 years were found and analyzed. Mean age was 37.6 ± 11.8 years; 37 patients were women. At VA-ECMO implantation, cardiogenic shock was present in 38 patients, cardiac arrest in 12, and severe hemodynamic instability in 7. A peripheral approach was used with 47 patients, whereas 10 patients had a central implantation or other access. RESULTS: Mean VA-ECMO support was 9.9 ± 19 days (range, 2 to 24 days). Cardiac recovery with ECMO weaning was achieved in 43 patients (75.5%), major complications were observed in 40 patients (70.1%), and survival to hospital discharge occurred in 41 patients (71.9%). After hospital discharge (median follow-up, 15 months) there were 2 late deaths. The 5-year actual survival was 65.2% ± 7.9%, with recurrent self-recovering myocarditis observed in 2 patients (at 6 and 12 months from the first AFM event), and 1 heart transplantation. CONCLUSIONS: Cardiopulmonary support with VA-ECMO provides an invaluable tool in the treatment of AFM, although major complications may characterize the hospital course. Long-term outcome appears favorable with rare episodes of recurrent myocarditis or cardiac-related events.

Video assistance in mitral surgery: reaching the "Thru" port access.

BACKGROUND: Minimally invasive and video assisted mitral valve surgery has been used widely since beginning of 20th. Different reduced surgical approaches allowed replacing or repairing a mitral valve sparing sternal incision. Nevertheless the most used strategy has been in the last years the right mini thoracotomy and the extra thoracic cardiopulmonary bypass (CPB). The main goal is avoiding sternal approach for mitral valve procedures and improve postoperative course of the patients. Some postoperative complication likes blood loss, need for transfusion, prolonged intubation and infection has been reduced using this alternative technique. A special advantages has been reported in elderly or high risk patients and in redo cases. METHODS: Several cardiac centres using videoscopy and a revolutionary set up for CPB management and aortic occlusion have adopted the approach. The team approach, including surgeon, anaesthesiologist, nurse, cardiologist and perfusionist, is crucial for a safe and effective realization of this surgical strategy. The proper use of catheters and Seldinger skilfulness, and the guidance of trans-esophageal echocardiography (TEE) during the procedure are two milestones of this technique. A careful and progressive learning curve is required for all the components of the team. In fact some peculiarity likes modified surgical instruments, 3D and Full HD video assisted view, percutaneous canulation for CPB and myocardial protection, etc., make this procedure challenging for all members of the operative room (OR) team. RESULTS: Our favourite set-up include right mini thoracotomy in the IV intercostal space, femoral vein and arterial canulation and an additional venous cannula in the superior vena cava for the drainage of the upper part of the body. Aortic occlusion is achieved usually using an endo-aortic clamp positioned by means of continuous and careful TEE guidance. A mitral valve procedure is realized by direct or video guided view; using adapted and shaft instruments or special atrial retractors all standard techniques are used in this setting. CONCLUSIONS: The literature reports and our published results showed the technique is safe, easy to replicate and allows an excellent rate of valve repair even in more complex patients.