Heart Transplantation Following Fontan Failure: Long-Term Survival Analysis.

Objectives: Fontan circulation presents significant challenges for patients with congenital heart disease, often necessitating heart transplantation (HTX) due to deteriorating functionality across multiple organ systems. However, the impact of prior Fontan palliation on HTX outcomes remains poorly understood, with early mortality rates suggesting a heightened risk. The aim of our study is to evaluate the long-term results after heart transplantation in patients with univentricular congenital heart disease previously palliated with Fontan circulation. Methods: A retrospective analysis was conducted on patients who underwent HTX for congenital heart disease. Patients were categorized into two groups based on the pre-HTX circulation pathway: the Failing Fontan Group (FFG) and the Biventricular Congenital Group (BCG). Data were collected from patients between 1987 and 2018. Early and late outcomes, including survival rates, were assessed and critically analyzed. Results: Of the 66 patients, 29 (43%) had a failing Fontan palliation (FFG), and 37 had biventricular congenital diseases (BCG) before heart transplantation. Early mortality (30-day) was not statistically different between the two group. The overall survival rate was 82.6 ± 13.9% at 1 year, 79.0 ± 14.9% at 5 years, 67.2 ± 17.6% at 10 years and 63.2 ± 18.2 ± at 15 years for the FFG, and 86.1 ±11.4% at 1 year, 79.5 ± 13.7% at 5 years, 75.7 ± 14.9% at 10 years, 75.7 ± 14.9% at 15 years for the BCG, with no statistically significant difference (Mantel Cox p value: 0.69, 0.89, 0.52 and 0.39, respectively). Regarding Cox-regression analysis, the long-term survival rate was not affected either by previous Fontan surgery or by the era of heart transplantation (before vs. after the year 2000). Conclusions: Although heart transplantation after Fontan palliation showed a higher risk in the early post-operative period, the medium- and long-term survival rates are comparable with biventricular circulation patients. Despite the failing Fontan patients being a challenging set of candidates for transplantation, it is a reasonable option in their treatment.

Impact of the coronavirus disease 2019 (COVID-19) pandemic on the Italian congenital cardiac surgery system: a national survey.

OBJECTIVES: Italy has been one of the countries most severely affected by the coronavirus disease 2019 (COVID-19). The Italian government was forced to introduce quarantine measures quickly, and all elective health services were stopped or postponed. This emergency has dramatically changed the management of paediatric and adult patients with congenital heart disease. We analysed data from 14 Italian congenital cardiac surgery centres during lockdown, focusing on the impact of the pandemic on surgical activity, patients and healthcare providers and resource allocation. METHODS: Fourteen centres participated in this study. The period analysed was from 9 March to 4 May. We collected data on the involvement of the hospitals in the treatment of patients with COVID-19 and on limitations on regular activity and on the contagion among patients and healthcare providers. RESULTS: Four hospitals (29%) remained COVID-19 free, whereas 10 had a 39% reduction in the number of beds for surgical patients, especially in the northern area. Two hundred sixty-three surgical procedures were performed: 20% elective, 62% urgent, 10% emergency and 3% life-saving. Hospital mortality was 0.4%. Compared to 2019, the reduction in surgical activity was 52%. No patients operated on had positive test results before surgery for severe acute respiratory syndrome coronavirus 2, the virus responsible for COVID-19. Three patients were infected during the postoperative period. Twenty-nine nurses and 12 doctors were infected. Overall, 80% of our infected healthcare providers were in northern centres. CONCLUSIONS: Our study shows that the pandemic had a different impact on the various Italian congenital cardiac surgery centres based on the different patterns of spread of the virus across the country. During the lockdown, the system was able to satisfy all emergency clinical needs with excellent results.

Sutureless aortic valve replacement in high risk patients neutralizes expected worse hospital outcome: A clinical and economic analysis.

BACKGROUND: Aortic valve replacement (AVR) by sutureless prostheses is changing surgeon options, although which patients benefit most, as well as their possible economic impact is still to be defined. METHODS: Perceval-S prosthesis (LivaNova) is reserved, at the documented Institution, for patients at perceived high surgical risk. This retrospective analysis of outcome and resource consumption compared Perceval with other tissue valves. To clarify the comparison, only patients respecting 'instructions-for- use' of Perceval were reviewed. INCLUSION CRITERIA: > 65 years, +/- coronary artery bypass grafting, patent foramen ovale closure or myectomy. EXCLUSION CRITERIA: bicuspid, combined valve or aortic sur- gery. Costs were calculated per patient on a daily basis including preoperative tests, operating costs (hourly basis), disposables, drugs, blood components and personnel. RESULTS: The sutureless group (SU-AVR) had a higher risk profile than the sutured group (ST-AVR). Cardiopulmonary bypass (CPB) and cross-clamp times were significantly shorter in SU-AVR (isolated AVR: cross-clamp 52.9 ± 12.6 vs. 69 ± 15.3 min, p < 0.001; CPB 79.4 ± 20.3 vs. 92.7 ± 18.2 min, p < 0.001). Hospital mortality was 0.9% in SU-AVR and nil in ST-AVR, p = 0.489; intubation 7 (IQR 5-10.7) and 7 h (IQR 5-9), p = 0.785; intensive care unit 1 (IQR 1-1) and 1 day (IQR 1-1), p = 0.258; ward stay 5.5 (IQR 4-7) and 5 days (IQR 4-6), p = 0.002; pacemaker 5.7% (6/106) and 0.9% (1/109), p = 0.063, respectively. Hospital costs (excluding the prosthesis) were $12,825 (IQR 11,733-15,334) for SU-AVR and $12,386 (IQR 11,217-14,230) in ST-AVR, p = 0.055. CONCLUSIONS: Despite higher operative risks in SU-AVR, hospital mortality, morbidity and resource consumption did not differ. Operative times were shorter with the sutureless device and this improve- ment, along with more frequent ministernotomy, may have improved many postoperative aims.

Sutureless prosthesis for failed small Mitroflow valves: the Perceval-after-Mitroflow procedure.

Because of its favourable haemodynamic characteristics and easy implantability, Mitroflow aortic valve bioprosthesis has been the valve of choice for many surgeons in patients with small aortic annulus. Disappointingly, early structural valve deterioration and high transvalvular gradients have been reported mostly in older patients with small prostheses. Reimplanting a new stented prosthesis sutured in a narrow and damaged annulus is technically challenging and demanding mainly in high-risk patients. Valve-in-valve transcatheter aortic valve implantation has been proposed as a viable option; however, it presents significant limitations because of residual high transprosthetic pressure gradients and risk of coronary occlusion. We report a series of 8 patients, with medium-term follow-up, who underwent successful Perceval-S surgical sutureless aortic implant after the removal of a degenerated small Mitroflow valve. No early mortality occurred, but 1 patient died 4 months postoperatively due to gastrointestinal disease. No major complications occurred. Early and mid-term postoperative pressure gradients were low (mean gradients 13.1 ± 3.3 mmHg and 10.2 ± 3.8 mmHg, respectively). In operable patients with a degenerated Mitroflow valve, these favourable clinical and haemodynamic results suggest that the sutureless solution is a simple, valid and safer alternative to conventional redo valve replacement or to valve-in-valve transcatheter aortic valve implantation.

Perceval valve-in-valve implant for full root xenograft failure.

Aortic root replacement with a biological conduit results in excellent hemodynamics but has limited durability. We report a series of six patients who underwent successful Perceval-S surgical aortic valve implantation following surgical deterioration of a biological valve composite conduit and discuss the technical details and the importance of appropriate valve sizing for this procedure.

Time series analysis of physiologic left ventricular reconstruction in ischemic cardiomyopathy.

OBJECTIVE: The history of left ventricular reconstruction has demonstrated that the full spectrum of recoverable physiologic parameters is essential for a good functional result. We report the long-term outcome of a new surgical technique that arranges myocardial fibers in a near-normal disposition, also recovering left ventricular twisting. METHODS: Between May 2006 and October 2013, 29 consecutive patients with previous anterior myocardial infarction and heart failure symptoms underwent physiologic left ventricular reconstruction surgery and coronary revascularization. Patients were examined by means of standard echocardiography and 2-dimensional speckle tracking at 8 time steps until 7 years after surgery. Ten geometric and functional parameters were evaluated at each step and analyzed by the linear mixed model test. RESULTS: Hospital mortality was 0%. The mean percentage of indexed end-diastolic and end-systolic volume reduction was 45.7% and 50.9%, respectively. Ejection fraction and all of the volumes were significantly different in the postoperative period with a steady correction during time. Diastolic parameters were not worsened by surgical reconstruction. Ejection fraction and deceleration time showed a significant improvement during time. Left ventricular torsion increased immediately after the surgical correction from 2.8 ± 4.4 degrees to 8.7 ± 3.9 degrees (P = .02) and was still present 4 years after surgery. CONCLUSIONS: Surgical conduction of ventricular reconstruction should be standardized to achieve the full spectrum of recoverable physiologic parameters. The renewal of ventricular torsion should be pursued as an adjunctive element of ventricular efficiency, mainly in ventricles that work at a critical level in the Frank-Starling relationship and pressure-volume loop.

Perceval sutureless valve in freestyle root: new surgical valve-in-valve therapy.

Aortic root replacement with biological conduit (homograft, autograft, or xenograft) is a valuable tool, but biological valves are often prone to degeneration. Reoperations usually require root removal and repetition of the Bentall procedure to maintain the same orifice area. A less radical option is to limit replacement to the valve. In cases of calcified or very small roots, standard valve implantation cannot be performed, and bailout with a sutureless valve may be a particularly useful option. Here we have described a case of leaflet rupture in a calcified small Freestyle root (Medtronic Inc, Minneapolis, MN) in which we performed a valve-in-valve (V-in-V) procedure with a Perceval-S prosthesis (Sorin Group, Saluggia, Italy).

Medium-term results of systematic off-pump coronary surgery performed by trainee surgeons.

OBJECTIVE: Our unit has used off-pump coronary artery bypass (OPCAB) surgery since 1998, and has consequently developed teaching methods for surgical trainees. This study aimed to compare the medium-term results of OPCAB performed by experts or supervised trainees. METHODS: We retrospectively analysed the data relating to 1333 OPCAB operations performed between January 1998 and January 2006 (mean patient age: 65.3 + or - 13; M/F ratio: 2.9), and compared the medium-term outcomes of the 977 (73.3%) carried out by three expert surgeons (group A) with the remaining 356 (26.7%) carried out by four supervised trainees (group B). RESULTS: There were no preoperative differences in patient age, gender, angina class, operative priority, extent of coronary artery disease, the presence of a recent myocardial infarction or left main stenosis or European System for Cardiac Operative Risk Evaluation (EuroSCORE) between the two groups. Thirty-day mortality was 1% in group A and 0.6% in group B (p=0.43), and 4-year actuarial survival, respectively, 97.4 + or - 1.1% and 94.3 + or - 4.1% (p=0.41); the freedom from new re-vascularisation rates in the two groups were, respectively, 96 + or - 0.7% and 95.3 + or - 1.4% (p=0.3). CONCLUSIONS: The results of this study reflect our unit's long experience of OPCAB surgery and that its successful re-engineering towards the systematic use of OPCAB was feasible. They also show that, in this context, teaching OPCAB surgery is safe in a non-selected cohort of patients, and that the medium-term outcomes of the patients operated on by trainee or expert surgeons are similar.

'Let's twist again': surgically induced renewal of left ventricular torsion in ischemic cardiomyopathy.

OBJECTIVES: To test the potential of the heart to be surgically restored at a near-normal global condition, granted that its physiological characteristics are respected (working volumes, chamber geometry, fiber orientation, opposite rotation of apex and base, global torsion and strain). METHODS: From May 2007 to December 2008, 12 consecutive patients with ischemic cardiomyopathy were included in this study. All patients underwent modified surgical anterior ventricular restoration combined with complete coronary revascularization and, when indicated, mitral anuloplasty. The modified restoration aims to re-approach residual myocardium, redirecting fiber orientation displaced by infarct scar toward a more physiological gross disposition. Patients were studied preoperatively and postoperatively with a complete echocardiographical assessment, including speckle-tracking analysis. RESULTS: Standard parameters significantly improved after the operation (end diastolic volume, P < 0.001; end systolic volume, P < 0.001; ejection fraction, P = 0.004), and so did peak systolic apical rotation, peak systolic left ventricular torsion and two-chamber and four-chamber longitudinal strain (P = 0.004, 0.003, 0.05 and 0.01, respectively). Pearson's correlation between apical rotation and longitudinal strain (two-chamber and four-chamber) was -0.877 (P < 0.001) and -0.720 (P = 0.008), respectively, and between torsion and longitudinal strain was -0.845 (P = 0.001) and -0.785 (P = 0.002), respectively. CONCLUSION: This study reveals an unexpected potential of the myocardium to be restored at a near-normal global condition, with regard to all of its physiological characteristics. The concept of fiber-based surgical treatment, supported by an imaging-guided preoperative study, could widen the potential of repairing a failing heart.

A new no-touch aorta technique for arterial-source, off-pump coronary surgery.

The possibility of avoiding the manipulation of the ascending aorta plays a key role in the neuroprotective effect of off-pump coronary revascularization, reducing the overall invasiveness. We have devised a new surgical plan using the proximal stump of the right internal thoracic artery as an intrathoracic, arterial source of flow for the saphenous vein, avoiding direct aorta manipulation. The saphenous vein can be as long as required, and its proximal anastomosis guarantees a better match of the two conduits and undergoes a lower peak pressure.

Patch size, shape and orientation affect geometrical outcomes of surgical anterior ventricular restoration.

OBJECTIVES: To verify whether the use of a small, oval-shaped patch limits the trend toward re-dilatation compared to endoventricular circular patch plasty and leads to different geometrical and functional results in surgical anterior restoration. METHODS: Thirty-seven patients with ischemic cardiomyopathy after anterior myocardial infarction end-systolic volume index of > or =45 ml/m2, ejection fraction of < or =35%, and no combined mitral procedures, underwent surgical anterior ventricular restoration between January 2000 and April 2003: 18 patients (group 1) were operated on using the endoventricular circular patch plasty technique (mean patch area 9.6 cm2) and 19 patients (group 2) received a small, obliquely oriented, oval-shaped patch (mean patch area 6.2 cm2). Ten geometrical parameters were studied preoperatively and at least 6 and 12 months after surgery. Data were analyzed using repeated-measures ANOVA, chi2, paired and unpaired Student's t-test, and binary logistic regression. RESULTS: Group 1 showed a worsening over time in systolic and diastolic longitudinal length, end-diastolic volume (P < 0.001), end-diastolic volume index (P = 0.006), end-systolic volume (P = 0.005), and end-systolic volume index (P = 0.03). Group 2 showed an improvement in percentage of akinesia and wall motion score index (P < 0.001) and a worsening only in end-systolic diameter (P = 0.03) and end-diastolic volume (P = 0.04). At 12-month follow-up, ANOVA revealed that the oval patch positively influenced end-diastolic volume (P = 0.03), end-systolic volume (P = 0.03), and end-systolic volume index (P = 0.05), and group 2 had a significantly higher number of patients with an end-systolic volume index of <45 ml/m2 (P = 0.01). CONCLUSION: The use of a small, narrow, obliquely oriented, oval patch may help to prevent adverse ventricular remodeling over time.

A new shape for an old function: lasting effect of a physiologic surgical restoration of the left ventricle.

BACKGROUND: Long-term morphofunctional outcome may vary widely in surgical anterior left ventricular wall restoration, suggesting variability in post-surgical remodeling similar to that observed following acute myocardial infarction. The aim of this pilot study was to demonstrate that surgical restoration obtained with a particular shape of endoventricular patch leads to steady morphofunctional ventricular improvement when geometry, volume and residual akinesia can be restored as normal as possible. METHODS: This study involved 12 consecutive patients with previous anterior myocardial infarction, dilated cardiomyopathy and no mitral procedures, who underwent left ventricular reconstruction and coronary revascularization between May 2002 and May 2003 using a small, narrow, oval patch aiming at a volume

Factors affecting left ventricular remodeling after valve replacement for aortic stenosis. An overview.

Although a small percentage of patients with critical aortic stenosis do not develop left ventricle hypertrophy, increased ventricular mass is widely observed in conditions of increased afterload. There is growing epidemiological evidence that hypertrophy is associated with excess cardiac mortality and morbidity not only in patients with arterial hypertension, but also in those undergoing aortic valve replacement. Valve replacement surgery relieves the aortic obstruction and prolongs the life of many patients, but favorable or adverse left ventricular remodeling is affected by a large number of factors whose specific roles are still a subject of debate. Age, gender, hemodynamic factors, prosthetic valve types, myocyte alterations, interstitial structures, blood pressure control and ethnicity can all influence the process of left ventricle mass regression, and myocardial metabolism and coronary artery circulation are also involved in the changes occurring after aortic valve replacement. The aim of this overview is to analyze these factors in the light of our experience, elucidate the important question of prosthesis-patient mismatch by considering the method of effective orifice area, and discuss surgical timings and techniques that can improve the management of patients with aortic valve stenosis and maximize the probability of mass regression.

Effect of the increase in valve area after aortic valve replacement with a 19-mm aortic valve prosthesis on left ventricular mass regression in patients with pure aortic stenosis.

OBJECTIVES: Although patients with aortic stenosis, who receive 19-mm valves, are at high risk for prosthesis-patient mismatch, most of them show a significant left ventricular mass (LVM) regression postoperatively. The aim of this study was to identify factors predicting postoperative relative LVM regression in this subgroup of patients. METHODS: A population of 44 patients operated on for pure aortic stenosis and receiving a 19-mm valve was studied by echocardiography at 1.4 +/- 0.5 years postoperatively. RESULTS: The mean relative LVM regression was -19.3 +/- 18.9%, the mean gradient drop was -31.6 +/- 13.3 mmHg, and the mean Delta increase in aortic area index (postoperative aortic area index minus preoperative aortic area index) was 0.30 +/- 0.14 cm/m. Thirty-two patients had an indexed effective orifice area of less than 0.8 cm/m. At multivariate analysis (r = 0.63; r = 40%; P < 0.0001) preoperative LVM (P = 0.006), hypertension (P = 0.018) and Delta aortic area index (P = 0.049) were independent predictors of relative LVM regression. CONCLUSIONS: Our study shows that, at least 1 year postoperatively, in patients receiving a 19-mm valve, LVM regression is influenced by several parameters, in particular preoperative LVM, hypertension and the magnitude of the increase in aortic area.

Impact of prosthesis-patient mismatch on cardiac events and midterm mortality after aortic valve replacement in patients with pure aortic stenosis.

BACKGROUND: Prosthesis-patient mismatch (PPM) occurs when the effective orifice area (EOA) of the prosthesis being implanted is too small in relation to body size, thus causing abnormally high transvalvular pressure gradients. The objective of this study was to examine the midterm impact of PPM on overall mortality and cardiac events after aortic valve replacement in patients with pure aortic stenosis. METHODS AND RESULTS: The indexed EOA (EOAi) was estimated for each type and size of prosthesis being implanted in 315 consecutive patients with pure aortic stenosis. PPM was defined as an EOAi < or =0.80 cm2/m2 and was correlated with overall mortality and cardiac events. PPM was present in 47% of patients. The 5-year overall survival and cardiac event-free survival were 82+/-3% and 75+/-4%, respectively, in patients with PPM compared with 93+/-3% and 87+/-4% in patients with no PPM (P< or =0.01). In multivariate analysis, PPM was associated with a 4.2-fold (95% CI, 1.6 to 11.3) increase in the risk of overall mortality and 3.2-fold (95% CI, 1.5 to 6.8) increase in the risk of cardiac events. The other independent risk factors were history of heart failure, NHYA class III-IV, severe left ventricular hypertrophy, and absence of normal sinus rhythm before operation. CONCLUSIONS: PPM is an independent predictor of cardiac events and midterm mortality in patients with pure aortic stenosis undergoing aortic valve replacement. As opposed to other risk factors, PPM may be avoided or its severity may be reduced with the use of a preventive strategy at the time of operation.

Staged correction of double discordance, situs inversus, and absence of true pulmonary arteries.

The case of a young boy with an extremely rare and complex cardiac malformation is described. Situs inversus, dextrocardia, double discordance with pulmonary atresia, absence of true pulmonary arteries, and systemico-pulmonary collateral arteries was the original diagnosis. Three preparatory operations were necessary to reconstruct and unifocalize the pulmonary vascular tree. At the age of 7 years complete correction with intraoperative stenting of the left neopulmonary artery was successfully performed. After more than 3 years from the definitive operation, the patient is alive with no physical limitations.

Impact of the improvement of valve area achieved with aortic valve replacement on the regression of left ventricular hypertrophy in patients with pure aortic stenosis.

BACKGROUND: Previous studies have reported that patient-prosthesis mismatch may be associated with lesser regression of left ventricular hypertrophy. However, among the patients with mismatch, the extent of left ventricular mass regression varied markedly from one patient to another, and we hypothesized that it could be related to the magnitude of the increase in valve area achieved with aortic valve replacement. Our aim was to examine the relationship between the improvement in valve effective orifice area obtained with aortic valve replacement and the extent of postoperative left ventricular mass regression in patients with patient-prosthesis mismatch. METHODS: Preoperative and postoperative measurements of valve effective orifice area, transvalvular pressure gradient, and left ventricular mass were obtained by Doppler echocardiography in 111 patients with pure aortic stenosis who were found to have patient-prosthesis mismatch based on an indexed effective orifice area of 0.8 cm2/m2 or less after operation. RESULTS: On average, the valve effective orifice area increased by 0.45 +/- 0.24 cm2/m2 with operation, and mean transvalvular pressure gradients decreased by -31.6 +/- 13.5 mm Hg. The absolute and relative differences between preoperative and postoperative left ventricular mass were -28 +/- 30 g and -17% +/- 18%, respectively. In multivariate analysis, higher preoperative left ventricular mass (p < 0.0001) and larger increase in indexed valve effective orifice area with operation (p = 0.019) were independently associated with greater left ventricular mass regression. CONCLUSIONS: This study shows that in patients with patient-prosthesis mismatch, the degree of left ventricular mass regression is influenced by the increase in valve effective orifice area achieved by aortic valve replacement.

Impact of valve prosthesis-patient mismatch on left ventricular mass regression following aortic valve replacement.

BACKGROUND: Valve prosthesis-patient mismatch is a frequent problem in patients undergoing aortic valve replacement and its main hemodynamic consequence is to generate high transvalvular gradients through normally functioning prosthetic valves. The persistence of high gradients may hinder or delay the regression of left ventricular hypertrophy after aortic valve replacement. METHODS: The aim of the study was to determine the impact of prosthesis-patient mismatch on the postoperative regression of left ventricular mass. Left ventricular mass was measured by Doppler echocardiography in 109 patients undergoing aortic valve replacement with a single type of bioprosthesis (Carpentier-Edwards Perimount) for pure aortic stenosis. Prosthesis-patient mismatch was defined as a projected indexed effective orifice area less than 0.90 cm2/m2. On this basis, 58/109 (53.2%) patients had prosthesis-patient mismatch. RESULTS: There was a good correlation (r = 0.61, p < 0.001) between the postoperative mean transprosthetic gradient and the projected indexed effective orifice area. The absolute and relative left ventricular mass regression was significantly (p = 0.002 and p = 0.01, respectively) lower in patients with prosthesis-patient mismatch (-48 +/- 47 g, -17% +/- 16%) compared to those with no prosthesis-patient mismatch (-77 +/- 49 g, -24% +/- 14%). In multivariate analysis, a larger projected indexed effective orifice area, female gender and a higher preoperative left ventricular mass are independent predictors of greater left ventricular mass regression. CONCLUSIONS: This study shows that in patients with pure aortic stenosis prosthesis-patient mismatch is associated with lesser regression of left ventricular hypertrophy after aortic valve replacement. These findings may have important clinical implications given that prosthesis-patient mismatch is frequent in these patients.