Long-term outcomes of right ventricle-to-pulmonary artery conduit insertion in adults with congenital heart disease: survival analysis by National Death Index.

OBJECTIVES: The aim of this study was to investigate the long-term outcomes following right ventricle-to-pulmonary artery (RV-to-PA) conduit insertion of Medtronic Freestyle® porcine valve (MFV) or pulmonary allograft valve (PAV) in adult patients with congenital heart disease. METHODS: Retrospective medical record review of consecutive RV-to-PA conduit insertion, using either PAV or MFV from 1991 to 2017. Perioperative data and clinic reports were collected. Cause and date of death were obtained from the Australian National Death Index to obtain survival function. RESULTS: In total, 232 patients (median age 31.5 years, interquartile range 25-41 years) underwent RV-to-PA conduit insertion (PAV = 84 and MFV = 148) and were eligible for inclusion [63.8% tetralogy of Fallot (TOF); 11.6% congenital pulmonary stenosis (PS); 24.6% other diagnoses]. The overall median follow-up time was 9.1 years (interquartile range 5.3-12.6 years). The mean gradient was 11.8 ± 7.1 mmHg in PAV and 16.6 ± 9.6 mmHg in MFV patients. Congenital PS patients had 100% survival at 20 years, TOF patients at 5, 10, 15 and 20 years had 99%, 97%, 96% and 96% survival, respectively. Patients with other primary diagnoses at 5, 10, 15 and 20 years had 93%, 91%, 87% and 87% respectively. Freedom from reintervention did not differ significantly at 5 and 10 years between pulmonary allograft (98.6%, 98.6%) and Freestyle® porcine bioprosthesis (97.5%, 93%). CONCLUSIONS: Both valves perform equally well with regard to patients' freedom from reoperation, although transvalvular gradient was higher for Freestyle® patients. Congenital PS and TOF patients had better survival than patients with other primary diagnoses.

DCD donations and outcomes of heart transplantation: the Australian experience.

PURPOSE: There is increasing clinical utilization of hearts from the donation after circulatory death (DCD) pathway with the aim of expanding the donor pool and mitigating the ever-present discrepancy between the inadequate availability of good quality donor hearts and the rising number of patients with end-stage heart failure. METHODS: This article reviews the rationale, practice, logistical factors, and 5-year experience of DCD heart transplantation at St Vincent's Hospital, Sydney. FINDINGS: Between July 2014 and July 2019, 69 DCD donor retrievals were undertaken resulting in 49 hearts being instrumented on an ex situ normothermic cardiac perfusion device. Seventeen (35%) of these hearts were declined and the remaining 32 (65%) were used for orthotopic DCD heart transplantation. At 5 years of follow-up, the 1-, 3-, and 5-year survival was 96%, 94%, and 94% for DCD hearts compared with 89%, 83%, and 82% respectively for donation after brain death (DBD) hearts (n.s). The immediate post-implant requirement for temporary extra-corporeal membrane oxygenation (ECMO) support for delayed graft function was 31% with no difference in rejection rates when compared with the contemporaneous cohort of patients transplanted with standard criteria DBD hearts. SUMMARY: DCD heart transplantation has become routine and incorporated into standard clinical practice by a handful of pioneering clinical transplant centres. The Australian experience demonstrates that excellent medium-term outcomes are achievable from the use of DCD hearts. These outcomes are consistent across the other centres and consequently favour a more rapid and wider uptake of heart transplantation using DCD donor hearts, which would otherwise be discarded.

Early Outcomes of Surgery for Carcinoid Heart Disease.

BACKGROUND: The prognosis of patients with metastatic neuroendocrine neoplasms (NEN) continues to improve with modern oncological therapy. In the subgroup of patients with carcinoid syndrome, the development of carcinoid heart disease (CaHD) severely impacts long term survival. Valve surgery has been demonstrated to improve survival and symptoms in patients with CaHD. We sought to assess the outcomes of surgery for CaHD from a single Neuroendocrine Service. METHODS: We retrospectively reviewed outcomes of patients with CaHD and metastatic NEN who underwent valvular surgery over a 4-year period (2012-2016). RESULTS: Twenty (20) patients (mean age 64 years, range 29-77 years), all with metastatic small intestinal NEN treated with somatostatin analogues, underwent surgery. Tumour grade was: G1 (n=8), G2 (n=9), and unknown (n=3). Preoperative New York Heart Association (NYHA) class was III/IV in 15 patients (75%). The valves affected were: tricuspid (n=20; 19 replace, 1 repair), pulmonary (n=14; 14 replace), mitral (n=2; two replace) and aortic valve (n=2; two replace). Concomitant procedures included patent foramen ovale closure (n=9), right ventricular outflow tract (RVOT) (n=4) augmentation and coronary artery bypass grafting (n=3). There were two operative deaths (10%) due to right heart and liver failure. At 6 weeks, all surviving patients had symptom improvement (NYHA I/II). Median follow-up was 2±1.5 years (<1 month to 5 years). One asymptomatic patient developed RVOT obstruction after pulmonary replacement. Two patients had valvular recurrence. One and 2-year survival were 74% and 48% respectively. Of 13 late deaths, 12 were cancer-related (two with uncorrected progressive pulmonary regurgitation). CONCLUSIONS: Surgery for CaHD can be performed with satisfactory early results, leading to an improvement of cardiac symptoms, survival and enabling subsequent oncologic treatment. Further studies are required to improve longer term outcomes in these complex patients with CaHD.

Use of Transcatheter Valves for Open Surgical Mitral Valve Replacement. Australian Experience.

BACKGROUND: Mitral valve procedures remain a surgical challenge in the presence of extensive annular calcification, which presents a formidable technical challenge. Aggressive debridement is limited by risk of serious complications and the technical complexity of pericardial patch reconstruction of the debrided area. METHODS: An open surgical approach with a transcatheter valve allows the valve to be placed under direct visualisation to facilitate positioning and to evaluate the likelihood of both perivalvular leakage and atrioventricular disruption. The open approach has the additional advantage of performing concomitant surgeries like other valve procedures, arrhythmias surgeries and coronary bypass. RESULTS: We present our experience with open surgical mitral valve replacement (MVR) using transcatheter valve in different patients requiring varied procedures. These patients were not suitable for MVR using standard prosthetic valve and techniques. They were also not suitable for percutaneous MVR because of heavily calcified anterior mitral leaflet and the other concomitant procedures required. CONCLUSIONS: Open MVR with a transcatheter balloon-expandable valve can avoid the need for technically challenging and high-risk decalcification of mitral annulus. These novel techniques using transcatheter valves can be successful in complex cases where standard prosthetic valves are impossible to implant in a heavily calcified mitral annulus.

Surgical Aortic Valve Replacement Following Early Sapien-XT Valve Failure: A First.

BACKGROUND: Early degeneration of prosthetic aortic valve in transcatheter aortic valve replacement (TAVR) is a rare complication. METHOD: We report the case of a 75-year-old woman who presented with severe calcific stenosis of Edwards SAPIEN-XT valve implanted only 4 years previously. She is a Jehovah's Witness and has background of Sjogren's syndrome with secondary cryoglobulinaemic vasculitis. She was not a suitable candidate for valve-in-valve TAVR in view of early prosthetic valve failure by calcification. RESULT: She underwent surgical explantation of the Sapiens XT valve and mechanical aortic valve replacement. CONCLUSION: To our knowledge, this is the first time that early TAVR valve degeneration and failure of an Edwards SAPIEN-XT valve was reported which required surgical replacement with a mechanical valve.

Giant solitary fibrous tumor of the pleura.

Solitary fibrous tumors are rare mesenchymal tumors accounting for <5% of all neoplasms in the pleura and other sites. A 45-year-old man reported to us with cough and dyspnea. Radiological investigations revealed a giant mass displacing the mediastinum to the left. The tumor weighing 3.0 kg was successfully resected via a right thoracotomy. Histology and immunohistochemistry confirmed a benign tumor. Recurrence and malignant transformation of these benign tumors have been reported. Our patient has been followed up for 4 years with no recurrence.

Unusual case of chondroblastic osteosarcoma of the rib in an adult.

Primary osteosarcoma arising from the ribs is very rare. An unusual case of a primary chondroblastic osteosarcoma of the rib in a 30-year-old woman is described here. The patient underwent wide excision of the tumor along with the involved rib, followed by chemotherapy. Although clinically unsuspected in this unusual site, pathology confirmed a completely resected chondroblastic osteosarcoma.