Pulmonary arterial hypertension associated with congenital heart disease: lessons learnt from the large Turkish Nationwide Registry (THALES).

Pulmonary hypertension is a group of diseases, including pulmonary arterial hypertension associated with congenital heart disease (APAH-CHD), characterized by progressive deterioration in pulmonary hemodynamics associated with substantial morbidity and mortality risk. THALES is a national multicenter, prospective observational registry, providing data on patients with APAH-CHD. The study comprised APAH-CHD patients (>3 months of age) with confirmed diagnosis of right heart catheterization or echocardiographic findings. Initial and follow-up data were collected via regular hospital visits. Descriptive statistics are used for definitive purposes. Overall, 1034 patients aged 3 months-79 years (median 11.2 [Q1-Q3: 2.2-24.3] years) with APAH-CHD were enrolled at 61 centers, 50.3% being retrospectively enrolled. Most had either Eisenmenger's syndrome (49.2%) or systemic-to-pulmonary shunts (42.7%). Patients were mostly in functional class I-II at the time of diagnosis (46.6%). Mean 6-min walk distance (6MWD) was 369 ± 120 m. Mean pulmonary arterial pressure was 54.7 ± 22.2 mmHg for the whole group, and was highest in patients with Eisenmenger's syndrome. Targeted therapies were noted in 398 (38.5%) patients (monotherapy in 80.4%). Follow-up data were available in 506 patients. Survival at 140 months was 79% and was associated with baseline 6MWD >440 m (p = 0.009), brain natriuretic peptide level < 300 ng/L (p < 0.001). Follow-up 6MWD >165 m (p < 0.0001), brain natriuretic peptide level <300 ng/L (p = 0.031), and targeted therapies (p = 0.004) were also predictive of survival. THALES is the largest registry dedicated to APAH-CHD to date and provides important contributions on demographics, clinical characteristics, and gaps in disease management.

Open hemiarch versus clamped ascending aorta replacement for aortopathy during initial bicuspid aortic valve replacement.

BACKGROUND: There is controversy regarding the extent of aortic resection necessary in patients with aortopathy related to bicuspid aortic valve disease. To address this issue, we reviewed our experience in patients undergoing ascending aorta replacement during bicuspid aortic valve replacement. METHODS: We reviewed 702 patients who underwent ascending aorta replacement at the time of initial nonemergent native bicuspid aortic valve replacement at our institution between January 2000 and June 2017. Treatment cohorts included an open hemiarch replacement group (n = 225; 32%) and a clamped ascending aorta replacement group (n = 477; 68%). RESULTS: Median patient age was 60 years (interquartile range [IQR], 51-67 years), female sex was present in 113 patients (16%), ejection fraction was 62% (IQR, 56%-66%), and aortic arch diameter was 33 mm (IQR, 29-36 mm). Cardiopulmonary bypass time was longer in the hemiarch replacement group (188 minutes vs 97 minutes; P < .001). Procedure-related complications (36%) and mortality (<1%) were similar in the 2 groups; however, the hemiarch group had an increased odds of blood transfusion (odds ratio, 1.62; 95% confidence interval [CI], 1.15-2.28; P = .006). The median duration of follow-up was 6.0 years (95% CI, 5.3-6.8 years). Overall survival was 94 ± 1% at 5 years and 80 ± 2% at 10 years. Multivariable analysis demonstrated similar survival in the 2 groups (hazard ratio, 0.83; 95% CI, 0.51-1.33; P = .439). No repeat aortic arch operations were done for aortopathy over the duration of clinical follow-up. CONCLUSIONS: Compared with patients in the clamped ascending aorta replacement group, patients in the hemi-arch replacement group had longer cardiopulmonary bypass and aortic cross-clamp times, along with an increased risk of blood transfusion, but similar freedom from repeat aortic arch operation and survival. We identified no advantage of performing hemiarch replacement in the absence of aortic arch dilation.

A Case Series of Long-Term Surgical Outcomes of Primary Pulmonary Artery Sarcomas With Opportunities for 3D-Printed Models in Surgical Planning.

There are limited data regarding the surgical management of primary pulmonary artery sarcomas (PPAS) because of their rarity and complicated diagnostic history. The objective of this study was to analyze our institution's long-term surgical management outcomes for PPAS in the absence of a care pathway. From May 1997 to June 2013, 8 patients (mean age 60.6 ± 11.8 years; range, 40-73 years; 5 women and 3 men) underwent surgical intervention for PPAS at our institution. The most common computed tomography finding was a luminal filling defect obstructing the pulmonary artery (PA), without evidence of extraluminal extension. Three patients underwent debulking/pulmonary endarterectomy alone and 5 patients underwent a more radical resection with PA patch angioplasty, PA resection and reconstruction, pulmonary valve replacement, and unilateral pneumonectomy. The mean postoperative survival in this series was 3.8 ± 3.6 years (range, 1-11.9 years), with 2 radical surgical resection patients alive at 4.9 and 11.9 years, respectively. For those patients with incomplete resection, 3-dimensional (3D) models were created to demonstrate the advantage of a preoperative guide for a more complete resection and what it would entail. Six patients had local recurrences with mean disease-free interval of 14 ± 10.9 months (range, 2 months-2.5 years), and 2 patients with re-resections had an overall postoperative survival of 2.8 and 11.9 years, respectively. In our small cohort of PPAS, patients treated with radical surgical resection had better survival. The small number of PPAS cases in this series makes proving this association unlikely but warrants consideration.

Does Mitral Valve Calcium in Patients Undergoing Mitral Valve Replacement Portend Worse Survival?

BACKGROUND: Mitral annular calcification (MAC) is associated with worse outcomes after mitral valve replacement (MVR). With limited data available on long-term outcomes, we reviewed our experience of MVR in presence of MAC. METHODS: A retrospective review of 1,710 consecutive patients who underwent MVR between January 2000 and December 2015 was performed. Patients with isolated primary MVR (n = 496) were included, whereas patients with concomitant cardiac surgery (n = 1,068), previous MVR (n = 110), and mitral valve (MV) endocarditis (n = 36) were excluded. MV calcification was classified as MAC present in anterior/posterior annulus and vertically at the level of leaflets/subvalvular apparatus. A conservative approach towards annular debridement was followed. RESULTS: Our sample's mean age was 64.4 ± 14.1 years, and included 279 (56%) women. MV calcification was observed in 169 (34%) patients with MAC in 115 (23%). Older age, higher ejection fraction, peripheral vascular disease, diabetes, dialysis, and previous aortic valve surgery were associated with increased prevalence of MAC. Patients with MV calcification had higher stroke rate (p = 0.040), patients with anterior leaflet and commissural calcification had higher pacemaker implantation (p = 0.010, p = 0.001, respectively), and patients with circumferential MAC had higher postoperative dialysis (p = 0.006). Operative mortality was not significantly different (p = 0.466) between MAC (n = 1, 1%) and non-MAC (n = 9, 2%) patients. MAC was associated with late mortality (unadjusted hazard ratio, 1.62; 95% confidence interval, 1.20 to 2.18), though on multivariable analysis age, diabetes, dialysis, hypertension, previous aortic valve surgery, previous coronary artery bypass grafting, and MVR with a bioprosthetic valve were found to be independent risk factors for mortality whereas MAC was not. CONCLUSIONS: A conservative approach to treat MAC achieves satisfactory results. Patients with MAC have significant comorbidities contributing to a worse survival, though MAC in itself is not a risk factor for mortality.

History of the World Society for Pediatric and Congenital Heart Surgery: The First Decade.

The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) is the largest professional organization in the world dedicated to pediatric and congenital heart surgery. The purpose of this article is to document the first decade of the history of WSPCHS from its formation in 2006, to summarize the current status of WSPCHS, and to consider the future of WSPCHS. The WSPCHS was incorporated in Canada on April 7, 2011, with a head office in Montreal, Canada. The vision of the WSPCHS is that every child born anywhere in the world with a congenital heart defect should have access to appropriate medical and surgical care. The mission of the WSPCHS is to promote the highest quality comprehensive cardiac care to all patients with congenital heart disease, from the fetus to the adult, regardless of the patient's economic means, with an emphasis on excellence in teaching, research, and community service.

The improvement of care for paediatric and congenital cardiac disease across the World: a challenge for the World Society for Pediatric and Congenital Heart Surgery.

The diagnosis and treatment for paediatric and congenital cardiac disease has undergone remarkable progress over the last 60 years. Unfortunately, this progress has been largely limited to the developed world. Yet every year approximately 90% of the more than 1,000,000 children who are born with congenital cardiac disease across the world receive either suboptimal care or are totally denied care.While in the developed world the focus has changed from an effort to decrease post-operative mortality to now improving quality of life and decreasing morbidity, which is the focus of this Supplement, the rest of the world still needs to develop basic access to congenital cardiac care. The World Society for Pediatric and Congenital Heart Surgery [http://www.wspchs.org/] was established in 2006. The Vision of the World Society is that every child born anywhere in the world with a congenital heart defect should have access to appropriate medical and surgical care. The Mission of the World Society is to promote the highest quality comprehensive care to all patients with pediatric and/or congenital heart disease, from the fetus to the adult, regardless of the patient's economic means, with emphasis on excellence in education, research and community service.We present in this article an overview of the epidemiology of congenital cardiac disease, the current and future challenges to improve care in the developed and developing world, the impact of the globalization of cardiac surgery, and the role that the World Society should play. The World Society for Pediatric and Congenital Heart Surgery is in a unique position to influence and truly improve the global care of children and adults with congenital cardiac disease throughout the world [http://www.wspchs.org/].