Tom Kai Ming Wang

Cystic fibrosis (CF) has undergone a remarkable transformation from a fatal childhood disease to a chronic adult condition, with a median predicted survival now exceeding 50 years following the introduction of highly effective CFTR modulator therapy (HEMT). This demographic shift has unmasked cardiovascular complications that were previously unrecognized due to premature mortality. In a multinational propensity-matched cohort, CF conferred a 65% higher risk of major adverse cardiac events (MACEs), including myocardial infarction, heart failure, and atrial fibrillation, versus matched controls (Frost et al., HR 1.65). In a separate Canadian population-based cohort, cardiovascular disease occurred at 2.9 times the rate of the general population and at least 20 years earlier (Arya et al., RR 2.9). Cardiac involvement extends beyond the traditional paradigm of cor pulmonale. CFTR is expressed in cardiomyocytes, vascular endothelium, and smooth muscle, where its dysfunction disrupts ion homeostasis, impairs nitric oxide-dependent vasodilation, and promotes oxidative stress. Clinically, these mechanisms manifest as biventricular dysfunction detectable by strain echocardiography and cardiac MRI even when ejection fraction is preserved, diffuse myocardial fibrosis, endothelial dysfunction and accelerated arterial stiffness from childhood, increased arrhythmia susceptibility, and, in a single-center cohort, an unexpectedly high prevalence of pericardial calcification (18%) on chest CT that awaits independent replication. Paradoxically, while HEMT has dramatically improved pulmonary outcomes, it introduces new cardiovascular considerations including hypertension, dyslipidemia, and obesity, and cross-sectional data in modulator-treated cohorts show persistent cardiac abnormalities, though whether these predate treatment or would differ without it is unknown. This review synthesizes the genetic, molecular, and clinical dimensions of cardiovascular disease in CF; distinguishes direct CFTR-mediated cardiac effects from indirect consequences of chronic lung disease; examines vascular, electrophysiological, valvular, and pericardial manifestations; and discusses management implications including CFTR modulator therapy, exercise, nutritional paradigm shifts, and transplantation considerations.

Objectives Characterisation of trends in acute coronary syndrome (ACS) outcomes are critical to informing clinical practice and quality improvement, but there are few recent population studies for ACS. We reviewed the recent trends in the outcomes of ACS in New Zealand (NZ). Methods All patients with ACS admitted to NZ public hospitals in 2006–2016 were identified from hospital discharge records, and their first ACS hospitalisations per year extracted for analysis. Thirty-day and 1-year death, myocardial infarction, stroke, heart failure and bleeding rates were calculated for each calendar year. Trends in outcome rates were assessed using generalised linear mixed models. Results Total annual ACS hospitalisations decreased from 685 to 424 per 100 000. Using first patient hospitalisations per year (n=1 55 060), we found significant annual declines in all major outcomes except for non-cardiovascular deaths. All-cause mortality fell from 10.5% to 9.1% at 30 days (adjusted OR 0.985 per year change, pConclusion ACS outcomes including all-cause mortality, cardiovascular death, myocardial infarction, stroke, heart failure and bleeding at 30 days and 1 year improved over the last decade in NZ, reflecting successful implementation and advances in prevention, medical and invasive management in ACS over time.

Background: We meta‐analyzed the efficacy and safety of catheter (CA) vs thoracoscopic (TA) ablation for atrial fibrillation (AF).Methods: PubMed, Embase, and Cochrane databases were searched until 31/12/2019 for relevant randomized trials and subsequent pooled analyses.Results: In six trials totaling 465 patients (254 CA/211 TA), 1‐year AF recurrences were higher for CA (46% vs 26%, odds ratio 2.90 [95% CI 1.32‐6.38]), whereas total adverse events were lower (10% vs 25%, 0.35 [0.14‐0.86], respectively).Conclusion: CA has lower efficacy but higher safety than TA. CA should remain the first‐line AF ablation strategy, and TA reserved for selected CA‐resistant patients where rhythm control is clinically necessary.

Background: International guidelines recommend early discharge for uncomplicated acute coronary syndrome (ACS) patients within 3 days; however, there is a paucity of contemporary literature regarding the safety of this strategy.Aims: To report the trends in the proportion of ACS hospitalisations discharged within 3 days and their outcomes in New Zealand.Methods: ACS hospitalisations 2006–2015 using national routinely collected data were categorised by length of stay (LOS) into ≤3, 4–5 and >5 days, excluding deaths during the index admission. Trend analysis of death, cardiovascular and bleeding events and their composites (net adverse clinical events) at 30‐day and 1‐year post‐discharge were performed using generalised linear mixed regression models adjusting for covariates by LOS subgroups.Results: Among 130 037 ACS hospitalisations, LOS ≤ 3 days increased from 32% in 2006 to 44% in 2016. This trend was observed for all demographics, ACS subtypes and management strategies. Event rates at 30 days and 1 year were the lowest for the LOS ≤3 days subgroup (all‐cause mortality 1.6% and 9.1% respectively). Thirty‐day and 1‐year all‐cause mortality rates were unchanged over time for this subgroup (adjusted odds ratio (95% confidence interval) of 1.011 (0.985–1.038) and 0.991 (0.979–1.003)), while net adverse clinical event rates significantly decreased (0.962 (0.950–0.973) and 0.972 (0.964–0.980) respectively).Conclusion: There was a substantial increase in early discharge post‐ACS over 10 years. These patients were associated with reduction in adverse clinical events up to 1 year and no increase in all‐cause mortality. These findings from a comprehensive national register suggest that guideline recommendations on early discharge after uncomplicated ACS are safe and appropriate.

BACKGROUND: Aortic dissection is a life-threatening condition frequently requiring emergency surgery. Key risk factors include hypertension and aortopathy syndromes; however, possible ethnic associations and differences in presentation and outcomes are less well established. We compared characteristics and outcomes of type A aortic dissection surgery by ethnicity. METHODS: Consecutive patients having type A aortic dissection surgery at Auckland City Hospital March 2003-March 2017 were divided into three ethnic groups: Maori, Pasifika and 'other', and analysed for characteristics, presentation and outcomes. RESULTS: Among 327 patients, 45 (14%) were Maori, 91(28%) were Pasifika Islander and 191 (58%) were other ethnicities. Mean age was lowest for Maori 51+/-12 years, then Pasifika 56+/-12 and other ethnicities 63+/-13 (P<0.001). Maori and Pasifika ethnicities had higher body mass index, more hypertension, dyslipidaemia and smoking, but lower proportion presenting in critical pre-operative state. Operative mortality occurred in 5 (11%), 18 (20%) and 42 (22%) for Maori, Pasifika and other ethnicities (P=0.258). Pasifika had higher age-standardised operative mortality standardised mortality ratio 6.00, 95% confidence interval 3.67-9.30 than 'other' ethnicities, while Maori had higher age-standardised late mortality 5.71, 2.90-10.2 respectively, and the latter association persisted in multivariable analysis. Critical pre-operative state and malperfusion syndrome independently predicted operative mortality. CONCLUSION: Maori and Pasifika patients were younger and present less unwell with type A aortic dissection, but had higher prevalence of cardiovascular risk factors. They had higher age-standardised late and operative mortality respectively, suggesting that aggressive management and risk factor control are critical for these patients.

Accurate risk stratification is important in pregnant women including the growing population with congenital heart disease (CHD). We meta-analysed the performance of risk models for CHD women. Six studies with 3426 pregnancies were studied, with cardiac complication rates of 6.7-20.6%. Pooled c-statistics (95% confidence interval) for scores were mWHO 0.71 (0.65-0.76), CARPREG 0.66 (0.61-0.71) and ZAHARA 0.71 (0.65-0.76). Current risk models had at best moderate discrimination for CHD women with significant room for improvement.