Autograft Reoperation Following the Ross Procedure: Evolving Strategies and Future Perspectives.

Background: Pulmonary autograft failure remains a cause of reoperation following the Ross procedure. The aim of this study is to describe our evolving approach to autograft reoperations. Methods: Retrospective study of all patients who underwent a pulmonary autograft reoperation following a Ross procedure between June 1997 and July 2022. Results: Two-hundred and thirty-five Ross procedures were performed. Thirty-six patients (15%) plus one referral underwent an autograft reoperation at a median of 7.8 years (IQR 4.6-13.6). The main indication was: neoaortic root dilatation associated with mild/moderate (n = 12) or severe (n = 8) aortic regurgitation; isolated severe aortic regurgitation (n = 6); infective endocarditis (IE) (n = 8); and aortic root pseudoaneurysm with no history of IE (n = 3). The autograft was spared in 29 cases (78%): 9 patients (24%) underwent aortic valve repair or aortic root remodeling, 15 patients (40%) aortic root reimplantation, 5 patients neoaortic root stabilization with a Personalized External Aortic Root Support (PEARS) sleeve. There were no in-hospital deaths. At a median follow up of 37 months (IQR 8-105), all patients were alive, 30 (81%) were asymptomatic. Eight patients (22%) required nine further reoperations. Estimated freedom from further reoperation was 90%, 72%, and 72% at 12-, 36-, and 60-months. Conclusions: Autograft reoperations following the Ross procedure can be safely performed and do not affect overall survival in the early and mid-term. Valve-sparing autograft replacement is technically feasible but remains at risk of further interventions. Alternative strategies, such as the PEARS sleeve, are becoming increasingly available but requires validation in the long term.

Total Aortic Arch and Neoaortic Root Replacement Following Stage III Palliation for Hypoplastic Left Heart Syndrome.

Progressive dilatation of the neoaortic root and reconstructed aortic arch is a serious complication after the Norwood procedure. There are no clear guidelines on the management of this complex anatomy in the setting of single ventricle physiology, and the surgical treatment of such an entity remains anecdotal. We describe a successful surgical repair in a 15-year-old girl presenting with a severely dilated neoaortic root and aortic arch causing compression and narrowing of the left pulmonary artery after successful three-stage palliation for hypoplastic left heart syndrome.

Outcomes of the Ross procedure in young adults with previous aortic valve interventions.

OBJECTIVES: Contemporarily modified Ross procedure continues to deliver excellent outcomes and remains part of the treatment strategy for aortic valve disease in the young adult population. The aim of this study was to assess whether Ross procedure carried out as a second or subsequent intervention for aortic valve disease carries similar risk and long-term benefit, when compared to Ross procedure for the first-time aortic valve replacement. METHODS: A total of 158 patients aged 16-60 years from a single congenital cardiac centre between 1997 and 2020 were included. The sample was split into 2 subgroups, based on the history of previous aortic valve interventions prior to the Ross procedure. Primary outcomes were defined as survival and pulmonary autograft failure. Coarsened exact matching was used to balance for covariates. RESULTS: A total of 103 patients underwent primary Ross and 55 underwent secondary Ross with a mean follow-up of 7.8 years. Twenty-two patients underwent 28 reoperations during follow-up. Forty-nine well-matched pairs were obtained through matching. Freedom from pulmonary autograft failure at 10 years was 84.5% in the primary group vs 100% in the secondary group (P = 0.021). Five- and ten-year survival were identical, 97.5% in the primary group vs 95.6% in the secondary group (P = 0.53). Male sex and era were found to be predictors of neoaortic root dilatation. CONCLUSIONS: The lower incidence of graft failure seen in secondary Ross could justify a stepwise approach to aortic valve intervention. Ross procedure delivers excellent outcomes in the adult population with no difference in survival for primary or secondary Ross.

Aortic root translocation and en bloc rotation of the outflow tracts surgery for complex forms of transposition of the great arteries and double outlet right ventricle: A multicenter study.

OBJECTIVE: There are several choices for the correction of complex transposition of the great arteries and double outlet right ventricle not amenable to the Rastelli-type surgery, but outcome data are limited to small series. This study aims to report results after the aortic root translocation and en bloc rotation of the outflow tract procedures. METHODS: This is a retrospective, multicentric, observational study. Clinical, anatomy, procedural, and detailed follow-up data (median, 4.43 years) were collected. RESULTS: A total of 70 patients (62.9% male; median age, 1 year; range 4 days to 12.4 years) were included: n = 43 in the aortic root translocation group and n = 27 in the en bloc rotation group. Those in the aortic root translocation group were older (P = .01) and more likely to have had previous procedures (P < .0001), but cardiac anatomy was similar in both groups. Aortic root translocation and en bloc rotation early mortality (30 days) was similar (4.7% vs 3.7%, P = .8). Late survival and freedom from any cardiac reintervention were 92.7% and 16.9% at 15 years overall, respectively. Freedom from right ventricular outflow tract/conduit reintervention was better in the en bloc rotation group than in the aortic root translocation group (100% vs 24.5%, P = .0003), but more patients in the en bloc rotation group had moderate (or worse) aortic valve regurgitation during follow-up (16% vs 2.6%, P = .07). CONCLUSIONS: Both aortic root translocation and en bloc rotation are valuable surgical options for the treatment of complex transposition of the great arteries and double outlet right ventricle. In the en bloc rotation group, there was better freedom from right ventricular outflow tract reinterventions, but a higher probability of aortic valve regurgitation. Identifying the main driving forces for these observed differences requires further study of these procedures.

Evolving Approach in Hypoplastic Left Heart Syndrome With Restrictive and Intact Septum.

Background: Hypoplastic left heart syndrome (HLHS) with either intact atrial septum (IS) or highly restrictive interatrial communication (HRIC) is associated with poor survival. Immediate postpartum access to cardiac therapy and timely left atrial decompression (LAD) are paramount to a successful outcome. We describe herein our evolving approach to LAD and report interstage and longer-term results. Methods: We retrospectively identified neonates with HLHS IS/HRIC requiring LAD between 2005 and 2019. All babies had prenatal/postnatal echocardiography. Our LAD strategy evolved over time from attempt at transcatheter balloon atrial septostomy (BAS) to surgical septectomy with inflow occlusion, to hybrid trans-atrial stent implantation. Results: Twelve neonates required LAD at a median time of 14 (0.5-31) hours after birth. Five patients underwent BAS that proved successful in 2 cases. Of the 3 unsuccessful cases, 2 required extra-corporeal membrane oxygenation (ECMO) support and died subsequently; one underwent hybrid trans-atrial stent implantation. Of the remaining 7 patients, 3 underwent surgical septectomy with inflow-occlusion and 4 underwent hybrid trans-atrial stent implantations. Overall, 8 patients survived LAD and reached Norwood palliation. Three of the 8 required ECMO postoperatively. There was no hospital mortality after Norwood stage 1 palliation and interstage survival was 100%. Six patients successfully underwent Glenn shunt (superior cavopulmonary anastomosis) and 5 have completed total cavopulmonary connection. Conclusions: Our experience suggests that prompt postnatal LAD can be safely achieved with careful multidisciplinary planning and accurate antenatal diagnosis. In our hands, hybrid trans-atrial septal stent insertion appears to be a safe approach which combines the versatility of transcatheter techniques together with the effectiveness of surgical control.

HeartWare Explant After Recovery 6 Years After Implant in a 3-Year-Old Child: Has the Game Changed?

The use of continuous-flow mechanical circulatory support in preschool children remains anecdotal. This case report describes the sequel to the implantation with a HeartWare HVAD system (Medtronic, Minneapolis, MN) in a 3-year old child. A 3-year-old boy with myocarditis-related cardiomyopathy underwent implantation with a HeartWare device. After an uncomplicated postoperative course, the patient was discharged home. Serial echocardiography showed progressive left ventricular recovery. After 6 months, the device was decommissioned, and the outflow graft was tied off. Six years after insertion, the device was explanted uneventfully. The HeartWare ventricular assist device offers viable long-term mechanical circulatory support in selected children that results in sustainable care and good quality of life.

Extracorporeal life support in the multidisciplinary management of cardiogenic shock complicating acute myocardial infarction.

OBJECTIVES: To analyze the results of extracorporeal life support (ECLS) for cardiogenic shock complicating acute myocardial infarction (AMI) in a single-center experience. BACKGROUND: Cardiogenic shock is still a leading cause of death for AMI. Conventional management carries mortality rates exceeding 50%. ECLS may be considered as a bridge to decision in the setting of AMI complicated by cardiogenic shock not responsive to standard management. METHODS: We performed an observational analysis of our local database. The primary end-point was survival to hospital discharge. All variables were compared between survivors and nonsurvivors. RESULTS: Between January 2007 and December 2017, 56 patients were supported for cardiogenic shock complicating AMI. The mean age was 56.7 years and 89.3% were males. Baseline characteristics were comparable between both groups. Of the 50 primary percutaneous coronary interventions that were attempted, 44 (88.0%) were successful. Twenty-three (41.1%) patients died during ECLS support. The complications' rate during ECLS support was comparable between both groups. Twenty-eight (50%) patients were successfully weaned from ECLS after a mean support of 8.7 days. Eight (14.3%) patients eventually died after weaning before hospital discharge. Five (8.9%) patients could not be weaned from ECLS and were switched to a long-term mechanical circulatory support. Overall survival to hospital discharge was 41.1% (n = 23). Eighteen (32.1%) patients were alive after a mean follow-up of 38.0 ± 29.9 (range, 4.2-95.4) months. CONCLUSIONS: ECLS should be considered as a therapeutic solution in the management of AMI-related cardiogenic shock with a satisfactory short- and long-term survival.

Reoperation for left atrioventricular valve failure in repaired atrioventricular septal defect: Can more valves be preserved in the current era?

OBJECTIVE: Left atrio-ventricular valve (LAVV) regurgitation after repair of an atrio-ventricular septal defect (AVSD) may necessitate further surgery. However, redo-LAVV repair remains challenging. We sought to determine if more LAVV valves are preserved in the current era, and analyze early and longer-term results. PATIENTS: All consecutive patients with repaired AVSD who underwent redo-LAVV surgery from January 2004 to April 2017 were included. Patients with single ventricles, atrial isomerism, and complex associated anomalies were excluded. METHODS: This was a single-center study using retrospective chart review and an institutional database for follow-up information. Data analyzed included number and year of primary AVSD and redo-LAVV operation, presence of trisomy 21, morphology of AVSD, mortality, and reoperation. Univariate analysis included repair and replacement rates and early and long-term survival. RESULTS: During the study period 36 redo-LAVV operations were performed, with repair in 28 and replacement in eight. The number of redo-operations increased from 13 in the first part to 23 in the second part of the study. The rate of LAVV preservation significantly increased over time (54% vs 91%, P < 0.01), and was not affected by morphology of AVSD or trisomy 21. There was one in-hospital death at Day 42 and overall estimated survival was 94.5% at 5 years. Freedom from reoperation after redo-LAVV repair was 87% at 5 years with no significant difference between repair and replacement groups. CONCLUSION: In the current era, more LAVVs can be preserved at the time of redo-operation with excellent early and long-term survival and acceptable reoperation rates. LAVV morphology and presence of trisomy 21 did not affect outcome.

Surgical rescue after transcatheter interventional procedures in congenital heart disease patients: an existing problem.

AIMS: Nowadays, transcatheter approaches are the treatment of choice for several congenital heart defects. However, adverse events may occur during interventional procedures. Even if the complication rate has been reduced remarkably because of learning curve and technological improvements, catastrophic events are still possible. The aim of this study was to review cardiac catheter complications that required surgical treatment during or after a percutaneous procedure. METHODS AND RESULTS: We evaluated retrospectively a thirteen-year experience at our centre. We examined all transcatheter procedures involving device release or implantation needing surgical rescue. We performed 3,205 interventional catheterisation procedures with device release or implantation: ASD device closure (n=2,205), PDA device occlusion (n=355), VSD device closure (n=218), aortic coarctation or recoarctation stenting (n=199), pulmonary artery stenting (n=154) and pulmonary valve implantation (n=74). Complications that required surgical treatment occurred in 1.2% of cases. Early surgery was performed in 22 cases, while in 18 patients a surgical treatment related to late complications was performed in a mean follow-up of 17 months. There were no deaths in either group. CONCLUSIONS: A spectrum of CHD can be treated today by transcatheter interventional procedures with good results and a low, but not negligible, risk of complications that require a surgical operation. The risk of developing late complications makes a long-term follow-up mandatory in such patients.

Long-term results of sequential vein coronary artery bypass grafting compared with totally arterial myocardial revascularization: a propensity score-matched follow-up study†.

OBJECTIVES: The aim of the study was to analyse the early and long-term outcomes of a consecutive series of patients who underwent sequential coronary artery bypass grafting (CABG) and to compare them with a matched population of totally arterial revascularized patients. METHODS: From January 1994 to December 1996, 209 patients underwent total arterial myocardial revascularization at our institution [arterial (ART) group]. In the same period, 2097 patients underwent CABG with left internal thoracic artery on left anterior descending and great saphenous vein on the right and circumflex branches sequentially [sequential vein (SV) group]. The propensity score methodology was used to obtain risk-adjusted outcome comparisons between the two groups (209 vs 243 patients in the ART group and SV group, respectively). RESULTS: In-hospital mortality was 1% in the ART group and 0.4% in the SV group (P = 0.86). Mean follow-up was 14 ± 4 years. Long-term survival was comparable among the two study groups [actuarial 5- and 15-year survival rates were 97 vs 93% and 82 vs 79% in the ART group and the SV group, respectively (P = 0.29)]. At follow-up, recurrence of angina (17 vs 18%; P = 0.99), acute myocardial infarction (MI) (3 vs 5%; P = 0.72) and repeated percutaneous coronary intervention (19 vs 21%; P = 0.69) were similar in the ART group compared with the SV group. In the Cox regression analysis, type of revascularization was not an independent predictor of any long-term outcomes (death or major adverse cardiac events). In asymptomatic patients, exercise stress test at follow-up was comparable between the two groups (P = 0.14). CONCLUSIONS: Sequential vein CABG appears to have good early and long-term clinical outcomes. Also, early and long-term incidence of acute MI was not significantly higher in the SV group. However, further studies with a larger population are warranted in order to confirm the present results.

Could anterior papillary muscle partial necrosis explain early mitral valve repair failure?

Standardized techniques of mitral valve repair (MVR) have recently witnessed the introduction of a 'respect rather than resect' concept, the strategy of which involves the use of artificial chordae. MVR displays several advantages over mitral valve replacement in degenerative mitral regurgitation (MR), but the risk of reoperation for MVR failure must be taken into account. Different mechanisms could be advocated as the leading cause of MVR failure; procedure-related mechanisms are usually involved in early MVR failure, while valve-related mechanisms are common in late failure. Here, the case is reported of an early failure of MVR using artificial chordae that could be explained by an unusual procedure-related mechanism, namely anterior papillary muscle necrosis. MVR failure is a well-known complication after surgical repair of degenerative MR, but anterior papillary muscle partial necrosis might also be considered a possible mechanism of procedure-related MVR failure, especially when considering the increasing use of artificial chordae. Owing to the encouraging results obtained, mitral valve re-repair might be considered a viable solution, but must be selected after only a meticulous evaluation of the underlying mechanism of MVR failure.

Quadricuspid aortic valve plasty: is it worth it to repair as an alternative to substitution?

We present the case of a 49-year-old man with an abnormal aortic valve formed by four cusps (three equal large cusps and one smaller cusp; type 1 according to Hurwits and Roberts classification) with a marked lack of coaptation that caused a severe aortic valve insufficiency. The patient underwent a repair of the defect with a tricuspidalization of the aortic valve, restoring the normal coaptation of the cusps and the normal function of the aortic valve. Postoperative course was unremarkable. The early follow-up showed a mild aortic valve insufficiency.

Pulmonary valve papillary fibroelastoma: management of an unusual, tricky pathology.

Papillary fibroelastomas are rare benign tumours of the endocardium, accounting for the most common primary valvular tumours of the heart. They typically originate from left-sided heart valves, whereas pulmonary valve involvement is anecdotal. They rarely cause valvular dysfunction, but they can cause turbulent flow and thrombus formation with consequent cerebral, retinal, coronary and pulmonary embolic disease and obstruction. We present here the case of a 56-year old man who was referred to our institution with an accidental finding, at transthoracic echocardiogram, of a mobile, pedunculated mass on the pulmonary valve, confirmed at cardiac magnetic resonance. He underwent surgical removal of the mass through median sternotomy with complete sparing of the valve. The postoperative course was unremarkable. Histopathological examination confirmed that the mass was a papillary fibroelastoma.

Aorta to right atrial shunt due to the rupture of a degenerative aneurysm of the noncoronary sinus of Valsalva.

Aneurysms of the sinus of Valsalva are a rare aortic pathology. Congenital aneurysms caused by weakness at the junction of the aortic media and the annulus fibrosus occur more commonly than acquired aneurysms, which are typically secondary to disease processes that involve the aortic root. The both can remain clinically silent for many years. About half are associated with aortic regurgitation. Symptoms can be caused by mechanical obstruction, with compression of the conducting system, or intracardiac rupture. Herein, the case of a 77 years old man, with no specific cardiologic risk factor, who came to our attention with an acute congestive heart failure and a diagnosis of a ruptured aneurysm of the noncoronary sinus of Valsalva is described. After diagnosis was confirmed, he underwent an emergency repair of the lesion.

Tracheostomy after cardiac operations: in-hospital and long-term survival.

BACKGROUND: Patients with ventilator dependency after cardiac operations may require a tracheostomy. This study determined the hospital and postdischarge outcome in a series of patients who underwent a tracheostomy due to ventilator dependency after cardiac operations. METHODS: Medical records of cardiac surgical patients were retrospectively reviewed for preoperative, intraoperative, and postoperative variables, and also prospective follow-up for all-cause mortality. All adult patients treated with tracheostomy due to postoperative ventilator dependency between January 1, 2004, and December 31, 2009, were admitted to the study. Statistical methods included a multivariable logistic regression analysis for hospital mortality and a Kaplan-Meier analysis with multivariable Cox regression model for postdischarge mortality. RESULTS: The study group included 131 patients. The hospital mortality rate was 49%. The only independent predictor for hospital mortality was the number of fresh frozen plasma units transfused during the hospital stay (6% mortality risk increase per each unit transfused). Survival rate for patients discharged from the hospital was 61% at 1 year, 49% at 2 years, 45% at 3 years, and 34% at 5 years. Factors affecting death after discharge were concomitant postoperative heart failure (hazard ratio, 2.6) and the length of ventilator dependency. CONCLUSIONS: Patients with a respiratory failure without associated heart failure or neurologic dysfunction have a significantly better long-term outcome. Caution should be applied in the use of fresh frozen plasma in patients with postoperative respiratory failure and ventilator dependency requiring a tracheostomy.