Surgical considerations for large asymptomatic cardiac fibromas in the context of fatal ventricular arrhythmias.

Cardiac fibromas are rare intracardiac neoplasms but represent the most common resectable tumours of childhood. They can remain asymptomatic for extended periods of time but a pertinent and unpredictable risk of sudden cardiac death and fatal ventricular arrhythmia always exists even in asymptomatic patients. We report a case of an asymptomatic two month-old with cardiac fibroma who presented with a ventricular tachycardia (VT) arrest. Here, we discuss management of cardiac fibroma in the context of occurrence of sudden VT.

Does patient gender affect outcomes after concomitant coronary artery bypass graft and aortic valve replacement? An Australian Society of Cardiac and Thoracic Surgeons Database study.

OBJECTIVES: Women undergoing isolated coronary artery bypass graft (CABG) surgery have been previously shown to be at an independently increased risk for post-operative morbidity and mortality. However, there are considerably less data on whether this trend remains true in patients undergoing concomitant aortic valve replacement (AVR) and CABG surgery. The aim of our study was to investigate this pertinent issue. METHODS: Data obtained between June 2001 and December 2009 by the Australasian Society of Cardiac and Thoracic Surgeons Cardiac Surgery Database Program were retrospectively analysed. Demographic, operative data and post-operative complications were compared between male and female patients using χ(2) and t tests. Long-term survival analysis was performed using Kaplan-Meier survival curves and the log-rank test. Independent risk factors for short- and long-term mortality were identified using binary logistic and Cox regression, respectively. RESULTS: Concomitant AVR and CABG surgery was undertaken in 2,563 patients; 31.8% were female. Female patients were older (mean age 76 vs. 73 years; p < 0.001) and presented more often with hypertension (p < 0.001) but less often with severely impaired ejection fraction (p < 0.001), peripheral vascular disease (p < 0.001) and triple vessel disease (p < 0.001). Women did not demonstrate an increased risk of 30-day mortality (4.8 vs. 3.3%) on univariate (p = 0.069) or multivariate (p = 0.236) analysis. Female gender was independently associated with post-operative myocardial infarction (p = 0.022) and red blood cell transfusion (p < 0.001). There was no difference in long-term survival between men and women on multivariate analysis (p = 0.413). CONCLUSION: Female gender is not associated with poorer short- or long-term outcomes after concomitant CABG and AVR surgery.

Long-term outcomes of single-ventricle palliation for pulmonary atresia with intact ventricular septum: Fontan survivors remain at risk of late myocardial ischaemia and death.

OBJECTIVES: The specific outcomes of patients with pulmonary atresia with intact ventricular septum late after Fontan palliation are unknown. Patients with smaller right ventricles and myocardial sinusoids are known to have worse survival in the first years of life. Whether the potential for coronary ischaemia affects the long-term outcomes of these patients after Fontan palliation is still unknown. METHODS: All patients with pulmonary atresia with intact ventricular septum who underwent the Fontan procedure from 1984 to 2016 in Australia and New Zealand were identified, and preoperative, perioperative and follow-up data were collected. RESULTS: Late follow-up data were available for 120 patients. The median length of follow-up after the Fontan procedure was 9.1 years (interquartile range 4.2-15.4 years). Late death occurred in 9% of patients (11/120). Six were sudden, unexpected deaths; 4 of those occurred in patients known to have right ventricle-dependent coronary circulation (RVDCC). Those with RVDCC had a higher incidence of sudden death (4/20 vs 2/100; P = 0.007). RVDCC was associated with late death (P = 0.01) and the development of myocardial ischaemia after Fontan completion (P < 0.001). The 10-year survival rate was 77% (95% confidence interval 56-100%) for patients with RVDCC vs 96% (95% confidence interval 92-100%) for patients without RVDCC. CONCLUSIONS: Long-term survival of patients with pulmonary atresia with intact ventricular septum after the Fontan procedure is excellent, but patients with RVDCC remain susceptible to coronary ischaemia and sudden death. Closer surveillance and investigation for exercise-induced ischaemia may be necessary.

Three decades later: The fate of the population of patients who underwent the Atriopulmonary Fontan procedure.

OBJECTIVE: To review our experience of patients with an atrio-pulmonary Fontan circulation to determine their long-term outcomes. METHODS AND RESULTS: A retrospective analysis of long-term follow-up data using the Australia and New Zealand Fontan Registry was performed. There were 215 patients surviving hospital discharge after an atrio-pulmonary Fontan completion. A total of 163 patients were alive at latest follow-up, with 52 deaths. Twelve patients had required heart transplantation and 95 had Fontan failure (death, transplantation, Fontan takedown, Fontan conversion, severe systemic ventricular dysfunction or NYHA≥3). Twenty-eight year freedom from death, death and transplantation and Fontan failure were 69% (95% CI 61-78%), 64% (95% CI 56-74%) and 45% (95% CI 36-55%) respectively. One hundred and thirty patients developed atrial arrhythmias. Freedom from arrhythmia at 28years post Fontan was 22.9% (95% CI: 15.1-30.8). Development of arrhythmia increased the likelihood of death (HR:2.97, 95%CI 1.50-5.81; p=0.002), death and heart transplantation (HR:3.11, 95%CI 1.64-5.87; p<0.001) and Fontan failure (HR:4.78 95%CI 2.95-7.74; p<0.001). There were 42 patients who had thromboembolic events, of which only 12 were therapeutically anti-coagulated. Two-thirds of the surviving patients (86/126) with an intact atrio-pulmonary Fontan were regularly reviewed. Patients receiving follow-up care with general cardiologists without specialised training were more likely to face Fontan failure than those managed by cardiologists with specialist training in congenital heart disease (HR: 1.94, 95% CI 1.16-3.24; p=0.02). The majority of the surviving patients (81/86) remained physically active and almost two-thirds (54/86) were currently employed. CONCLUSION: Two-thirds of the patients who underwent a classical atrio-pulmonary Fontan are still alive 3 decades later. The majority are affected by the burden of arrhythmias but remain functionally active today. These challenging patients should only receive follow-up care from cardiologists with specialised training.

Ten-year outcomes of Fontan conversion in Australia and New Zealand demonstrate the superiority of a strategy of early conversion.

OBJECTIVE: To investigate the benefits of a strategy of early Fontan conversion. METHODS: Using the Australia and New Zealand Fontan Registry, retrospective analysis of their long-term follow-up data was performed. RESULTS: Between 1990 and 2014, a total of 39 patients underwent surgical conversion in 6 centres at a median age of 23.8 years (IQR: 19.3-28.2), 18.7 ± 5.0 years post-Fontan. One centre tended to perform conversion earlier: interval since first documented arrhythmia 2.9 ± 4.0 vs 4.0 ± 4.2 years, average NYHA Class 2 ± 0.4 vs 3 ± 0.9 (P = 0.008), mean number of preop anti-arrhythmics 1 ± 0.4 vs 2 ± 0.6 (P = 0.05). Two patients underwent conversion to an extracardiac conduit only, while 36 had concomitant right atrial cryoablation, of which 16 also had pacemaker implantation. Nine patients suffered major cardiac-related complications (7 low output syndrome, 3 ECMO, 3 acute renal failure, one stroke) (2/17 from the early conversion centre and 7/22 of the others; P = 0.14). Four patients died in hospital (10.3%) and 4 late after a median of 0.9 years [95% confidence interval (CI): 0.5-1] after conversion. An additional 2 patients needed transplantation at 1 and 8.8 years after conversion, respectively. The 10-year freedom from heart transplantation was 86% (95% CI: 51-97%). Outcomes from the centre with an early conversion strategy were significantly better: 8-year freedom from death or heart transplantation was 86% (95% CI: 53-96) vs 51% (95% CI: 22-74; log-rank P = 0.007). Eight additional patients required pacemaker implantation and 5 had arrhythmia recurrence. CONCLUSIONS: Fontan conversion is associated with lasting survival outcomes up to 10 years after conversion. A strategy of surgical conversion at earlier stage of failure may be associated with better survival free from transplantation.

Barotrauma as aetiological cascade of fatal intrapulmonary plastic bronchitis in a post-Fontan child.

The role of barotrauma in the exaggeration of plastic bronchitis after Fontan circulation has yet to be examined. We aim to describe a case of a 4-year old post-Fontan circulation girl where barotrauma suffered during commercial air travel played a role in the aetiological cascade of plastic bronchitis.

Early and late outcomes after isolated aortic valve replacement in octogenarians: an Australasian Society of Cardiac and Thoracic Surgeons Cardiac Surgery Database Study.

OBJECTIVE: The advent of percutaneous aortic valve implantation has increased interest in the outcomes of conventional aortic valve replacement in elderly patients. The current study critically evaluates the short-term and long-term outcomes of elderly (≥80 years) Australian patients undergoing isolated aortic valve replacement. METHODS: Data obtained prospectively between June 2001 and December 2009 by the Australasian Society of Cardiac and Thoracic Surgeons National Cardiac Surgery Database Program were retrospectively analysed. Isolated aortic valve replacement was performed in 2791 patients; of these, 531 (19%) were at least 80 years old (group 1). The patient characteristics, morbidity and short-term mortality of these patients were compared with those of patients who were <80 years old (group 2). The long-term outcomes in elderly patients were compared with the age-adjusted Australian population. RESULTS: Group 1 patients were more likely to be female (58.6% vs 38.0%, p<0.001) and presented more often with co-morbidities including hypertension, cerebrovascular disease and peripheral vascular disease (all p<0.05). The 30-day mortality rate was not independently higher in group 1 patients (4.0% vs 2.0%, p=0.144). Group 1 patients had an independently increased risk of complications including new renal failure (11.7% vs 4.2%, p<0.001), prolonged (≥24 h) ventilation (12.4% vs 7.2%, p=0.003), gastrointestinal complications (3.0% vs 1.3%, p=0.012) and had a longer mean length of intensive care unit stay (64 h vs 47 h, p<0.001). The 5-year survival post-aortic valve replacement was 72%, which is comparable to that of the age-matched Australian population. CONCLUSION: Conventional aortic valve replacement in elderly patients achieves excellent outcomes with long-term survival comparable to that of an age-adjusted Australian population. In an era of percutaneous aortic valve implantation, it should still be regarded as the gold standard in the management of aortic stenosis.

Acute myocardial infarction in pregnant women.

Acute myocardial infarction (AMI) in pregnant women is a rare but potentially lethal occurrence that should be carefully managed, especially in consideration of cardiac conditions being a rising cause of maternal deaths. Risk factors for AMI occurrence, in addition to typical cardiac-related risk factors, include medical conditions such as (pre) eclampsia, blood transfusions, thrombophilia and postpartum infections. Being older, multigravida or in the third trimester of pregnancy is also associated with an increased risk. The pathophysiological causes underlying AMI in pregnancy are diverse but generally associated with the coagulative and physiological changes related to the pregnancy. The selection of diagnostic modality and treatment options require careful consideration for pregnancy-related changes as well as risk of harm to the patient and fetus. This paper serves to review available literature regarding an extensive range of management issues that directly impact on maternal and fetal outcomes.