Long-term Outcomes of the Ross Procedure for Young Patients with Aortic Valve Disease.

To define current role of the Ross operation in young patients, the outcome in those followed longer than 20 years were assessed. Between 1994 and 2020, 81 consecutive patients, 70 of 11 male and/or female, mean age 27 years underwent Ross procedure, accruing 20 years of follow-up or longer. Sixty-four had bicuspid valve (79%) and 54 (67%) aortic insufficiency, while 15 (19%) had undergone prior operations. Surgery consisted in root replacement in 53 patients, cylinder inclusion in 20 and sub-coronary graft in 8. There were 7 late deaths in 80 hospital survivors (median follow-up 21 years, IQR 20-23), with 88% ± 5% survival at 25 years. Thirty-four patients required left, 6 left and/or right and 1 right heart valve reoperation, on average 13 years after Ross procedure. Reoperation was valve-sparing in 18 (45%) patients and valve and/or root replacement in 22. Ten (24%) reoperated patients required a second reoperation 18 years after Ross procedure. Freedom from autograft reoperation was 46% ± 6%, while from autograft valve replacement was 60% ± 7%, thanks to autograft valve-sparing. Freedom from isolated right valve reoperation was 98% ± 4%. No mortality was associated with any of the 51 reoperations. Root technique was associated with reoperation (P = 0.024). Age at follow-up was 50 years (IQR 36-60), with 70 (96%) patients in NYHA class I and 6 (55%) women carrying out pregnancies. Young patients undergoing the Ross procedure enjoy unprecedented survival well into the third decade of follow-up, even when faced with reoperation. At 25 years risk of autograft reoperation is consistent, while negligible for homograft. Technical improvements at operation and valve-sparing at reoperation may prolong autograft valve durability.

Accuracy of Micro-Computed Tomography in Post-mortem Evaluation of Fetal Congenital Heart Disease. Comparison Between Post-mortem Micro-CT and Conventional Autopsy.

Aims: Early prenatal diagnosis of congenital heart disease is feasible. Conventional autopsy is the current gold standard method for post-mortem confirmation. Radiologic techniques alternative to conventional autopsy, such as post-mortem micro-computed tomography, have been proposed in case of limited diagnostic accuracy (i.e., early termination of pregnancy, samples of small dimension or of low weight). The aim of the present study was to define accuracy of micro-computed tomography for post-mortem diagnosis of congenital heart disease in gross anatomy samples. Methods and Results: Fetal heart underwent in-utero prenatal echocardiography and ex-vivo post-mortem evaluation by 9 µm resolution micro-computed tomography and conventional autopsy. For each case, 25 indices of cardiac anatomy were studied by post-mortem micro-computed tomography and conventional autopsy; these were used to compare the two post mortem techniques. Ten samples were examined (gestational age between 12 + 4 and 21 + 6 weeks of gestation). Considering comparable indices, agreement between post-mortem micro-computed tomography and conventional autopsy was of 100% and sensitivity and specificity were of 100%. In "challenging specimens," post-mortem micro-computed tomography diagnoses more indices as compared to conventional autopsy and 84% of "not-diagnostic" indices at conventional autopsy would be diagnostic at post-mortem micro-computed tomography. Conclusion: Micro-computed tomography can be a valid diagnostic alternative to conventional autopsy for post-mortem evaluation of human fetal heart. In addition, it may prove superior to conventional autopsy particularly in cases coming from early termination of pregnancy or in samples of small dimension or of low weight.

Three-Dimensional Printing of Fetal Models of Congenital Heart Disease Derived From Microfocus Computed Tomography: A Case Series.

This article presents a case series of n = 21 models of fetal cardiovascular anatomies obtained from post mortem microfocus computed tomography (micro-CT) data. The case series includes a broad range of diagnoses (e.g., tetralogy of Fallot, hypoplastic left heart syndrome, dextrocardia, double outlet right ventricle, atrio-ventricular septal defect) and cases also had a range of associated extra-cardiac malformations (e.g., VACTERL syndrome, central nervous system anomalies, renal anomalies). All cases were successfully reconstructed from the microfocus computed tomography data, demonstrating the feasibility of the technique and of the protocols, including in-house printing with a desktop 3D printer (Form2, Formlabs). All models were printed in 1:1 scale as well as with the 5-fold magnification, to provide insight into the intra-cardiac structures. Possible uses of the models include education and training.

A Low-Cost and Simple Genetic Screening for Cystic Fibrosis Provided by the Brazilian Public Health System.

Cystic fibrosis newborn screening was implemented in Brazil by the Public Health System in 2012. Because of cost, only 1 mutation was tested - p.Phe508del. We developed a robust low-cost genetic test for screening 11 CFTR gene mutations with potential use in developing countries.

Selective Cerebro-Myocardial Perfusion in Complex Neonatal Aortic Arch Pathology: Midterm Results.

Aortic arch repair in newborns and infants has traditionally been accomplished using a period of deep hypothermic circulatory arrest. To reduce neurologic and cardiac dysfunction related to circulatory arrest and myocardial ischemia during complex aortic arch surgery, an alternative and novel strategy for cerebro-myocardial protection was recently developed, where regional low-flow perfusion is combined with controlled and independent coronary perfusion. The aim of the present retrospective study was to assess short-term and mid-term results of selective and independent cerebro-myocardial perfusion in neonatal aortic arch surgery. From April 2008 to August 2015, 28 consecutive neonates underwent aortic arch surgery under cerebro-myocardial perfusion. There were 17 male and 11 female, with median age of 15 days (3-30 days) and median body weight of 3 kg (1.6-4.2 kg), 9 (32%) of whom with low body weight (<2.5 kg). The spectrum of pathologies treated was heterogeneous and included 13 neonates having single-stage biventricular repair (46%), 7 staged biventricular repair (25%), and 8 single-ventricle repair (29%). All operations were performed under moderate hypothermia and with a "beating heart and brain." Average cardiopulmonary bypass time was 131 ± 64 min (42-310 min). A period of cardiac arrest to complete intra-cardiac repair was required in nine patients (32%), and circulatory arrest in 1 to repair total anomalous pulmonary venous connection. Average time of splanchnic ischemia during cerebro-myocardial perfusion was 30 ± 11 min (15-69 min). Renal dysfunction, requiring a period of peritoneal dialysis was observed in 10 (36%) patients, while liver dysfunction was noted only in 3 (11%). There were three (11%) early and two late deaths during a median follow-up of 2.9 years (range 6 months-7.7 years), with an actuarial survival of 82% at 7 years. At latest follow-up, no patient showed signs of cardiac or neurologic dysfunction. The present experience shows that a strategy of selective and independent cerebro-myocardial perfusion is safe, versatile, and feasible in high-risk neonates with complex congenital arch pathology. Encouraging outcomes were noted in terms of cardiac and neurological function, with limited end-organ morbidity.

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 ±â€Š6 and 70 ±â€Š10%, 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.

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.

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.

Neonatal repair of truncus arteriosus with "scimitar-like" mixed total pulmonary venous return.

The association between truncus arteriosus and total anomalous pulmonary venous return is exceedingly rare. Here described is a neonate presenting with a common arterial trunk and a previously unreported "scimitar-like" mixed total pulmonary venous return who underwent a successful one-stage repair. The unprecedented anatomic pattern of the systemic and pulmonary venous connection and the original surgical strategy are discussed.

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.

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.

Comparison of the long-term survival benefits associated with revascularization or medical therapy in patients with known coronary artery disease undergoing transesophageal atrial pacing stress echocardiography.

Although the utility of stress echocardiography for the diagnosis and prognostic evaluation of patients with suspected coronary artery disease (CAD) has been widely reported, few studies have evaluated the role of revascularization in relation to the presence of inducible ischemia during stress in patients with known CAD. The study population consisted of 295 consecutive patients who underwent transesophageal atrial pacing stress echocardiography (TAPSE) in the echocardiographic laboratory of our division between January 1988 and September 1997, in whom coronary angiography was performed within 10 days of the test. Patients were then assigned to revascularization or medical treatment according to the treatment given within 60 days of TAPSE. Cardiac-related deaths were higher in medically treated (19 of 135) than in revascularized (8 of 160) patients (p = 0.03). Parameters measured with TAPSE, i.e., positivity of the test, change in wall motion score index (DeltaWMSI and peak WMSI) were significantly related to mortality in medically treated patients but not in revascularized patients. At multivariate analysis, DeltaWMSI remained the most powerful predictor of cardiac death in medically treated patients (p = 0.005). Mortality progressively increased with increments in extent of inducible ischemia among medically treated patients (5 of 71 patients in DeltaWMSI 0, 3 of 27 in DeltaWMSI 0 to 25, 11 of 37 patients in DeltaWMSI >25) but not among revascularized patients (3 of 58 patients in DeltaWMSI 0, 2 of 51 in DeltaWMSI 0 to 25, 3 of 51 patients in DeltaWMSI >25). The survival curve in medically treated patients with ischemia in a remote zone (24 patients, 8 deaths) was worse than in other groups of medically treated patients (41 patients, 6 deaths). In conclusion, in patients with known CAD, the presence and extent of ischemia as evaluated with TAPSE worsens survival, if revascularization is not performed. In patients without ischemia at TAPSE, revascularization or medical therapy are equally effective.