Endomyocardial fibrosis of the right ventricle: A case report of successful surgery.

AIMS: The case we report, shows a successful treatment of right ventricle endomyocardial fibrosis. MATERIALS AND METHODS: Surgical therapy by endocardial decortication seems to be beneficial for many patients with advanced disease who are in functional-therapeutic class III or IV. The operative mortality rate is high, but successful surgery has a clear benefit on symptoms and seems to favourably affect survival as well.

Pathoanatomic Findings and Treatment During Hypertrophic Obstructive Cardiomyopathy Surgery: The Role of Mitral Valve.

BACKGROUND: To assess the role of the mitral valve apparatus (leaflets, chordae and papillary muscles, (PM)) in left ventricle outflow tract (LVOT) obstruction, and results of the surgical treatment for hypertrophic obstructive cardiomyopathy (HOCM). METHODS: Twenty-eight consecutive patients (58±11years, 53% female) undergoing HOCM surgery from 2007 to 2016 at our institute were retrospectively reviewed. Endpoints included the involvement of the mitral valve in LVOT obstruction, mortality, and changes in clinical and echocardiographic characteristics after HOCM surgery. RESULTS: Secondary chordae tendineae tractioning the anterior mitral leaflet to the interventricular septum, and systolic anterior motion were detected in 78% of the patients. Anomalous, hypertrophied, and fused PM with muscularis trabeculae hypertrophy were found in 50%, 25%, and 35% of the patients, respectively. Four patients had posterior leaflet redundancy. Secondary chordae (92%), PM, and muscularis trabeculae resection (71%), and PM splitting and elongation (28%) were added variably to septal myectomy (100%). Nine procedures (32%) on mitral valve leaflets were performed, involving six posterior and three anterior mitral leaflets. Long-term follow-up was 4±2.8years. There was no hospital mortality, and NYHA was reduced from 3±0.5 to 1±0.7 (p<0.0001), the LVOT gradient from 88±35 to 20±18mmHg (p<0.0001), mitral valve regurgitation from grade 3±1 to 1±0.7 (p<0.0001), and septum thickness from 18±3 to 14±2mm (p<0.0001). CONCLUSIONS: The mitral valve apparatus contributes with all its components variably to LVOT dynamic obstruction thus surgical correction in addition to extended myectomy is recommended to achieve the best outcome.

Systemic or Endoventricular Thrombolysis to Treat HeartWare Left Ventricle Assist Device Thrombosis: A Clinical Dilemma.

Endoventricular thrombolytic procedure (ETP) has been used to treat continuous-flow left ventricle assist device (CF-LVAD) thrombosis. The study aims to investigate the occurrence of complications after ETP. Data were retrospectively reviewed and analyzed in a series of patients who underwent CF-LVAD followed by ETP. Since November 2010, 20 patients underwent HeartWare CF-LVAD implantation at our institute. Four patients (20%) developed pump thrombosis and underwent a total of nine ETPs with tissue plasminogen activator infused into the left ventricle. The mean age was 60.2 ± 9 years. ETP was performed via either the femoral (n = 6) or radial artery (n = 3). Five ETPs (55.5%) were complicated by left and right radial artery occlusion, two by groin hematomas, and one by femoral artery false aneurysm. ETP carries a strong risk of vascular access complications that, in CF-LVAD patients, may add to the already complex clinical profile and economic burden; thus, a less invasive treatment is advisable whenever required.

Septal Thickness Does Not Impact Outcome After Hypertrophic Obstructive Cardiomyopathy Surgery (Septal Myectomy and Subvalvular Mitral Apparatus Remodeling): A 15-Years of Experience.

Background: The aim of this study was to assess the impact of septal thickness on long-term outcomes of surgical treatment for hypertrophic obstructive cardiomyopathy (HOCM) and correction of mitral subvalvular anomalies. Methods: Sixty-six consecutive patients (58 ± 12 years, 56% female) undergoing extended septal myectomy and subvalvular mitral apparatus remodeling from 2007 to 2021 were retrospectively reviewed. Patients were divided into 2 groups according to septal thickness: moderate [< 18 mm, 29 patients (44%)] and severe [≥ 18 mm, 37 patients (56%)]. End points included survival, symptom improvement, reduction of left ventricle outflow tract (LVOT) gradient, resolution of mitral regurgitation (MR), and reoperation. Results: The mean interventricular septal thickness was 19 ± 3 mm, 15.8 ± 0.8 mm in patients with moderate and 21.4 ± 3.2 mm in those with severe hypertrophy. Preoperative data, intraoperative variables, postoperative complication rates, pre-discharge echocardiographic and clinical parameters did not differ between the two study groups [except for procedures involving the posterior mitral leaflet (p = 0.033) and septal thickness after myectomy (p = 0.0001)]. Subvalvular apparatus remodeling (secondary chordae of mitral valve resection and papillary muscle and muscularis trabecula procedures including resection, splitting, and elongation) was invariably added to septal myectomy (100%). Four (6%) procedures involved the posterior mitral leaflets. Mitral valve replacement was carried out in two patients (3%, p = 0.4). Reoperation for persistent MR was necessary in one patient (1%, p = 0.4). Neither iatrogenic ventricular septal defect nor in-hospital mortality occurred. During follow-up (mean 4.8 ± 3.8 years), two deaths occurred. NYHA class was reduced from 2.9 ± 0.7 to 1.6 ± 0.6 (p < 0.0001), the LVOT gradient from 89.7 ± 34.5 to 16.3 ± 8.8 mmHg (p < 0.0001), mitral valve regurgitation grade from 2.5 ± 1 to 1.2 ± 0.5 (p < 0.0001), and septal thickness from 18.9 ± 3.7 to 13.9 ± 2.7 mm (p < 0.0001). Conclusions: Regardless of septal thickness, subvalvular apparatus remodeling with concomitant septal myectomy can provide satisfactory long-term outcomes in terms of symptom improvement, LVOT obstruction relief, and MR resolution (without mitral valve replacement in most cases) in patients with HOCM.

[Surgical management of hypertrophic obstructive cardiomyopathy: the role of mitral valve]. / Trattamento chirurgico della cardiomiopatia ipertrofica ostruttiva: il ruolo della valvola mitrale.

Hypertrophic obstructive cardiomyopathy (HOCM) is the most frequently inherited cardiovascular disease (prevalence in the general population of 1/500) and is characterized by significant left ventricular hypertrophy, especially in the interventricular septum, combined with small-volume cardiac cavities. Transaortic surgical septal myectomy is the most commonly used technique to treat HOCM, and is associated with low operative morbidity and mortality and a reduction of the outflow gradients. The composite operative mortality of only 0.4% (17/3695 patients) from 5 major high-volume centers in North America highlights the role of dedicated HOCM units. The involvement of the mitral valve in the pathophysiology of HOCM has been addressed as systolic anterior motion (SAM)-related left ventricular outflow tract obstruction. Hypertrophic cardiomyopathy mitral malformations include leaflet elongation and a wide array of malformations of the papillary muscles and chordae that can be detected by transthoracic and transesophageal echocardiography and by cardiac magnetic resonance. Because they participate fundamentally in the predisposition to SAM, they have increasingly been repaired surgically.

Aortic surgery in Marfan patients with severe pectus excavatum.

AIMS: The optimal surgical management of the aortic root phenotype Marfan patients with severe pectus excavatum is a subject of debate. All the available literature were reviewed according to preferred reporting items for systematic reviews and meta-analyses (PRISMA) principles in order to assess the early outcomes of both pectus excavatum and aortic repair techniques. METHODS: Searches were done in PubMed and MEDLINE electronic databases dating from July 1953 to December 2015. RESULTS: A total of 97 peer-reviewed publications were retrieved, and 27 relevant publications were identified with a total of 39 Marfan patients with pectus excavatum who underwent ascending aorta and aortic root surgery. Emergency acute Type-A aortic dissection repair was reported in five cases. Concomitant pectus excavatum and aortic root repair and composite graft implantation were the most commonly performed procedures. Complications after a staged or a combined approach were uncommon and no deaths occurred. CONCLUSION: Aortic surgery in Marfan patients with pectus excavatum was carried out according to a variety of strategies, surgical techniques and accesses with low complications rate and no mortality. Many of these were well tolerated with minimal complications and no mortality.

Heart transplant program at IRCCS-ISMETT: Impact of mechanical circulatory support on pre- and post -transplant survival.

BACKGROUND: Heart transplantation (HTx) improves the quality of life and survival in patients affected by end-stage heart failure. The purpose of the current study is to present the patients' clinical data and results of HTx in a single Center of Sicily. Focus on survival after pre and post HTx mechanical circulatory support use will be performed. METHODS: 133 HTx were done from 2004 to the end of 2015.The average donor age was 34±13.5years and the proportion of male donors was 67%. Percentage of use of mechanical circulatory support to bridge patients to HTx was 18%. RESULTS: Overall pre-transplant mechanical circulatory support was not correlated to worse post-transplant prognosis, p=0.757. Severe primary early graft failure requiring extra corporeal membrane oxygenator support strongly impact the early mortality after heart transplantation (p<0.001). CONCLUSIONS: The results of HTx at ISMETT are comparable to those reported in high volume Italian transplant centers as well as in the ISHLT registry. The favorable outcome can be related to focus on multidisciplinary approach, strict recipients' selection and young donor population. Post HTx mechanical circulatory support use in general remains associated with worse post-transplant outcomes. This does not apply to pre-op mechanical circulatory support population.

Extracorporeal membrane oxygenation for graft failure after heart transplantation: a multidisciplinary approach to maximize weaning rate.

OBJECTIVES: Primary graft failure (PGF) after heart transplantation is a detrimental complication, and carries high morbidity and mortality. The aim of this study was to analyze the results of our multidisciplinary approach in supporting patients affected with PGF after heart transplantation. METHODS: Out of 114 consecutive patients receiving orthotopic heart transplantation between January 2006 and July 2013, 18 (15.7%) developed PGF requiring veno-arterial extracorporeal membrane oxygenator (VA-ECMO) support. Fourteen patients were male and the mean age was 49±11 years. General principles in treating the patients were based on a low dose of adrenaline (0.05 mic/kg per min) infusion; femoral intra-aortic balloon pump (13 of the 18 patients); low dose of vasoconstrictors; careful fluid balance; daily echocardiographic transesophageal monitoring. RESULTS: Mean graft recipient pulmonary vascular resistance was 3.6±3.2 WU. Five patients had absolute contraindication to IABP placement. The mean left ventricle ejection fraction pre-VA-ECMO was 18.4%±10.2%. The mean VA-ECMO and IABP support times were 6.7±3.2 and 9.2±7.6 days, respectively. Mean VA-ECMO flow was 4164±679 l/min. The mean left ventricle ejection fraction increased to 43.4%±17.7% at the end of support. Weaning and discharge rates in patients treated with VA-ECMO+IABP were 84% and 53%, respectively. Causes of death were primarily end-stage organ failure. CONCLUSIONS: A multidisciplinary evaluation of ECMO patients done by intensivists, cardiologists, and surgeons may influence weaning and survival rate. Our approach seems to be a safe and reproducible strategy for avoiding left ventricle distension and fluid overload, and for detecting complications that negatively affect outcomes.

Extracorporeal membrane oxygenator rescue and airborne transportation of patients with influenza A (H1N1) acute respiratory distress syndrome in a Mediterranean underserved area.

Adult respiratory distress syndrome (ARDS) secondary to H1N1 viral infection has been a worldwide medical and organizational challenge. We report our experience with extracorporeal membrane oxygenator (ECMO) rescue and transportation of patients with H1N1 ARDS within an insular and rural Mediterranean area of seven million inhabitants. A 24/7 on-call ECMO team was organized including one anesthesiologist, one cardiac surgeon, and one perfusionist. To limit missions' time to and from peripheral hospitals, airborne transportation with helicopter was the first choice. From November 2009 to January 2010, we performed 10 missions. Eight patients (80%) were placed on ECMO and transferred either on helicopter (70%) or with standard ambulance (10%). Average mission duration was nine hours (6-16 h). No complications secondary to the transportation means or to the ECMO were reported. Delivery of advanced medical technology can be achieved even in remote and underserved areas presenting geographical barriers. A multidisciplinary mobile ECMO team coordinated with adequate means of transportation could be routinely employed to rescue patients affected with other forms of severe acute hemodynamic and/or respiratory impairment.

Intraoperative validation of a new system for invasive continuous cardiac output measurement.

OBJECTIVE: Although bolus thermodilution technique for cardiac output (CO) measurement has widespread acceptance, new systems are currently available. We evaluated a continuous CO system (TruCCOMS, Aortech International Inc.) that operates on the thermal conservation principle and we compared it with the reference standard transit time flow measurement (TTFM). MATERIALS AND METHODS: Nine consecutive cardiac surgery patients were evaluated. After general anesthesia and intubation, a TruCCOMS catheter was percutaneously placed in the pulmonary artery (PA). After median sternotomy and pericardiotomy, a TTFM probe was placed around the main PA. Right ventricular (RV) CO measurements were recorded with both TruCCOMS and TTFM at different times: before cardiopulmonary bypass (CPB) (T0), during weaning from CPB (T1), and prior to sternal closure (T2). Data analysis included paired student t test, Pearson correlation test, and Bland-Altman plotting. RESULTS: TruCCOMS CO values were significantly lower at T0 (TruCCOMS 4.0 +/- 1.0 vs. TTFM 4.5 +/- 1.0 L/min; P < 0.0001) and T1 (TruCCOMS 3.6 +/- 0.5 vs. TTFM 4.2 +/- 0.7 L/min; P < 0.0001), and comparable at T2 (TruCCOMS 4.5 +/- 0.7 vs. TTFM 4.6 +/- 0.8 L/min; P = 0.4). Pearson test showed a significant correlation between TruCCOMS and TTFM CO measurements (RT0 = 0.9, RT1 = 0.8, RT2 = 0.6; P < 0.0001). Bland-Altmann plotting showed a bias of -0.53 +/- 0.43 L (-12%) at T0, -0.64 +/- 0.43 L (-14.5%) at T1, and -0.1 +/- 0.66 L (-0.8%) at T2. CONCLUSION: Although TruCCOMS may significantly underestimate CO, measurement trends correlate with TTFM. For this reason, a negative trend in RV output should trigger more specific diagnostic procedures.

Donor pharmacological hemodynamic support is associated with primary graft failure in human heart transplantation.

The aim of this study was to test the impact of donor and recipient characteristics on the development of primary graft failure (PGF) after heart transplantation (HT) by focusing on the donor's inotropic support. Heart donors and matched recipients data were prospectively collected. Univariate and multivariate analyses were used to determine independent predictors for PGF and peri-operative mortality. The donor's high inotrope requirement was defined as sustained need for dopamine exceeding 10 microg/kg/min and/or alpha agonists exceeding 0.06 microg/kg/min. PGF instead was defined as need for immediate post-HT mechanical circulatory support. Since 2006, we have performed 37 HTs. PGF occurred in six patients (16.2%). Although four patients (66.6%) were weaned off circulatory support, two of them (33.3%) died on mechanical assistance. Total in-hospital mortality was 10.8% (4/37). Upon multivariate analysis, pre-harvesting donor high inotrope dosage was the major determinant for PGF (P=0.03, OR=10.8). Given the organ shortage, many centers accepted marginal hearts assuming the donor's pre-harvest hemodynamic managing has a reduced impact on PGF development. As PGF remains the most lethal postoperative complication, the hazards should be carefully considered when using pre-harvesting high inotrope infusion rates.