Severe Systemic Ventricular Dysfunction Research Articles

Abstract 9972: Sex-Related Differences and the Influence of Pregnancy on Cardiac Outcomes in Adults With a Systemic Right Ventricle and Biventricular Physiology

Introduction: Transposition of the great arteries (TGA) with a systemic right ventricle (sRV) and biventricular physiology is associated with increased morbidity and mortality. There is a paucity of data regarding sex-related differences in outcomes in the context of a sRV. Moreover, pregnancy has been associated with deterioration of sRV function in short-term post-partum follow-up, but the long-term impact remains largely unknown. Methods and Results: A retrospective cohort study was conducted on 214 adults, age 44.7±12.3 years, with a sRV and biventricular physiology followed for a median of 13 years at an adult congenital heart disease center. No sex-related difference was identified in the prevalence of atrial or ventricular arrhythmias, permanent pacemaker implantation, hospitalization for heart failure, systemic atrio-ventricular valve intervention, heart transplant, or cardiac death. Among the 82 (38.3%) women, age 44.0±12.5 years, 43 (52.4%) had at least one full-term pregnancy. Women had a lower prevalence of moderate to severe sRV dysfunction than men (21% vs 42.6%, p=0.001) despite similar ages. Beta-blockers (p=0.008), furosemide (p=0.012), and mineralocorticoid receptor antagonists (p=0.028) were less frequently prescribed to women than men. Women had fewer implantable cardioverter-defibrillators (ICDs) for primary prevention than men (3.7% vs 13.6%, p=0.016), with no difference in the prevalence of secondary prevention ICDs (1.2% vs 2.3%, p=1). The four women with a prohibitive maternal mortality risk (modified WHO class IV) complied with recommendations to avoid pregnancy. After excluding these 4 women, no differences regarding frequency of adverse cardiac events, age at the time of event, and proportion with moderate or severe sRV dysfunction were observed in women with (N=43) and without (N=35) pregnancies during 14 years of follow-up. Conclusions: Women with TGA and a sRV had a lower prevalence of moderate to severe systemic ventricular dysfunction than men, along with a lower proportion of primary prevention ICDs. Following risk assessment and counselling with contraindication of pregnancy in the highest risk subgroup, pregnancy had no impact on long-term cardiac outcomes.

Initial Experience of Home Milrinone Therapy in Indian Setup: Case Series

<h3>Introduction</h3> Heart failure(HF) is a prevalent issue. Despite maximal oral medical therapy, rapid deterioration is known. Cardiac transplant remains the only definitive therapy. I/v/o limited available organs, patients face prolong waiting periods, clinical deterioration and high mortality. LVADs is not an option in resource-limited settings. Developed nations have used Long-term milrinone therapy in HF patients, but its utility is unexplored in our country. We report 3 cases with end-stage HF who were managed with long term milrinone therapy. One was successfully bridged while the other two are awaiting transplantation <h3>Case Report</h3> 1^st patient:- 37 year C-TGA+IVS +severe systemic AVVR. He presented to us with features of pulmonary oedema, severe systemic ventricular dysfunction and prior 3 episodes of HF requiring hospitalisation. 2^nd patient:-17 year old boy, with DCM in advanced HF. He presented with features of pulmonary oedema, severe systemic ventricular dysfunction, HF ACC stage D with organ dysfunction. 3^rd patient:- 12 year old girl, daughter of a single parent with ischemic cardiomyopathy due to LAD occlusion following MVR for MR(infective endocarditis) 6 months prior. She required ECMO postoperatively and had stormy post-op course(LVEF 45%). She was referred to us with acute HF and LVEF-10%. All were stabilized with non-invasive ventilation, Inj adrenaline, Milrinone and diuretics. Average ICU stay:-7 days. They continued to remain inotrope dependent(INTERMACS-3). They were listed for cardiac transplantation. They were discharged on home Milrinone infusion and anti failure medicines. At home, Milrinone was delivered through PICC line and battery-operated portable infusion pump.Patient-1 received Milrinone for 8 months while other 2 are on it since 4 months. None of them had further episodes of HF. All required PICC line change after 3 months.Patient-1 underwent successful transplantation after 8 months of listing. The other 2 are awaiting transplant <h3>Summary</h3> Utility of long term milrinone in a resource limited country like India is yet to be documented. The overall cost of home milrinone is less than cost of hospitalisation for recurrent HF episodes. Unavailability of home nursing care facility is a drawback. Long term Milrinone therapy is effective and can be safely used as a bridge to transplant. It is cost-effective, improves effort tolerance and reduces HF episodes

Cardiovascular outcomes in women with the highest classification of maternal cardiovascular risk in pregnancy

Abstract Introduction The modified world health organization (mWHO) classification of maternal risk is used to estimate morbidity and mortality in pregnant women with cardiovascular disease. Those in the highest risk category (mWHO Risk Class IV) are at greatest risk. Pregnancy is contraindicated in this patients. Methods This was a retrospective review of pregnant women classified as mWHO risk class IV, who were managed in a tertiary joint cardiac-obstetric pathway between 2008 and 2018. Results In total, there were 35 pregnancies in 30 women with the highest cardiovascular risk for adverse maternal outcomes. The mean maternal age at delivery was 29.3±5.2 years. Eleven (36%) patients were diagnosed with cardiovascular disease during pregnancy. Fourteen had a form of pulmonary arterial hypertension (46%), 6 (20%) had severe systemic ventricular dysfunction, 4 (13%) had severe mitral or aortic stenosis, 4 (13%) had aortic dilatation or inherited aortopathy, 1 (3%) had a history of peri-partum cardiomyopathy and 1 (3%) had severe native coarctation of the aorta. In the 30 pregnancies followed up in our centre, 29 (96%) were single foetus pregnancies and 1 (4%) was a twin pregnancy. There were 30 live births, 1 foetus was lost in the twin pregnancy. Of these 29 (96%) patients underwent elective caesarean section and 1 (4%) emergency caesarean section. Cardiovascular complications occurred in 18 (60%) women. Of these, 5 (28%) had atrial arrhythmias during pregnancy, 6 (33%) had worsening of pulmonary hypertension, 6 (33%) had decompensated heart failure. Three women had interventions during pregnancy: 1 had percutaneous intervention for coarctation of aorta due to foetal and maternal compromise, 1 had electrophysiological ablation for atrial arrhythmias to improve systemic ventricular function and 1 had an electrical cardioversion for atrial fibrillation. There were no deaths during pregnancy or in the peripartum period. One patient who presented at 34 weeks gestation with severe peripartum cardiomyopathy required early inotropic support followed by extracorporeal membrane oxygenation (ECMO) support post-delivery, died at 2 months post-partum. Conclusions With appropriate pre-pregnancy optimization, antepartum surveillance individualised peripartum care plans and multidisciplinary care throughout pregnancy, women at the highest risk for cardiovascular outcomes can have successful pregnancies, although the risk of cardiovascular complications remains high. Funding Acknowledgement Type of funding sources: None.

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

ObjectiveTo review our experience of patients with an atrio-pulmonary Fontan circulation to determine their long-term outcomes. Methods and resultsA 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. ConclusionTwo-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.

Current challenges in pediatric heart transplantation for congenital heart disease.

Pediatric heart transplantation is an established therapy for end-stage cardiac disease without suitable medical or surgical options. However, transplantation for congenital heart disease carries an incremental risk that challenges the pediatric transplant team on multiple levels. With improved outcomes following palliative and corrective congenital cardiac surgery, cardiac transplantation has decreased in recent years as a primary therapy. Nevertheless, congenital heart disease remains the most common indication for cardiac transplantation during infancy. Primary transplantation in infancy is selectively recommended for severe systemic ventricular dysfunction, severe atrioventricular valve insufficiency, and occlusive coronary artery anomalies, particularly with single ventricle physiology. Wait-list mortality remains highest for infants with prior palliative surgery and patients with failing Fontan physiology, both of whom have limited options for effective mechanical circulatory support. The sensitized patient carries an increased risk with prolonged wait times, although virtual cross-matches and single bead assays for donor-specific antigens have facilitated the transplant process. Early and late survival after transplantation for congenital heart disease remain inferior to cardiomyopathy, with prior Fontan procedure as a major risk factor. However, among survivors at 6 months, late outcomes are generally excellent. Major late causes of death include allograft vasculopathy, post-transplant lymphoproliferative disease, and acute rejection. Noncompliance with medications remains a major risk for teenage mortality. Despite the myriad of evolving challenges, pediatric heart transplantation for congenital heart disease enjoys routine short and long-term success at experienced centers for the vast majority of such patients without other options.

Preconception considerations

Heart disease complicates 1–2% of pregnancies, and in preparation for pregnancy, these women should receive comprehensive preconception counseling. Risks to the mother and potential risks to the offspring secondary to the type and severity of maternal cardiac disease, its inheritance pattern, required medical therapy, and palliative or corrective procedures that may be needed must be considered. Life expectancy and the ability to care for a child are somber issues that must be addressed when serious cardiac conditions exist. Pregnancy is still contraindicated in women with pulmonary hypertension, severe systemic ventricular dysfunction, dilated aortopathy, and severe left-sided obstructive lesions, but advances in medical and surgical management have resulted in an increasing number of patients with cardiac disorders who are interested in pursuing pregnancy. A multidisciplinary approach can best determine whether acceptable outcomes can be expected and what management strategies may improve the prognosis for women with heart disease and their offspring.

Sudden Cardiac Death in Adult Congenital Heart Disease

Sudden cardiac death (SCD) is a major cause of mortality in adults with congenital heart disease (CHD). The aim of this study was to determine the adult CHD population at risk of SCD and the clinical parameters associated with SCD. We performed a multicenter case-control study. Patients who died suddenly as a result of proven or presumed arrhythmia were included (cases). For each case, 2 controls matched on diagnosis, type of surgical intervention, age, and gender were included. From 3 databases including 25 790 adults with CHD, 1189 deaths (5%) were identified, of whom 213 patients (19%) died suddenly. Arrhythmic death occurred in 171 of 1189 patients. The underlying cardiac lesions were mild, moderate, and severe CHD in 12%, 33%, and 55% of the SCD cases, respectively. Clinical variables associated with SCD were supraventricular tachycardia (odds ratio [OR], 3.5; 95% confidence interval [CI], 1.5-7.9; P=0.004), moderate to severe systemic ventricular dysfunction (OR, 3.4; 95% CI, 1.1-10.4; P=0.034), moderate to severe subpulmonary ventricular dysfunction (OR, 3.4; 95% CI, 1.1-10.2; P=0.030), increased QRS duration (OR, 1.34 [per 10-ms increase]; 95% CI, 1.10-1.34; P=0.008), and QT dispersion (OR, 1.22 [per 10-ms increase]; 95% CI, 1.22-1.48; P=0.008). The clinical parameters found to be associated with SCD in adults with a broad spectrum of CHD, including systemic right ventricles, are similar to those in ischemic heart disease. Moreover, even those patients with mild cardiac lesions are potentially at risk for SCD. This highlights the need for further prospective studies as well as vigilant ongoing follow-up of the adult with CHD.

Maternal Cardiac Disease

Heart disease complicates more than 1% of pregnancies and is now the leading cause of indirect maternal deaths. The spectrum and severity of heart disease observed in reproductive-aged women is changing. Today, congenital heart disease accounts for more than half of cardiac disease in pregnancy, and ischemic heart disease is on the rise as a result of obesity, hypertension, diabetes, and delayed childbearing. Pregnancy is still contraindicated in women with pulmonary hypertension, severe systemic ventricular dysfunction, dilated aortopathy, and severe left-sided obstructive lesions, but advances in medical and surgical management have resulted in an increasing number of patients with congenital heart defects reaching childbearing age who are interested in pregnancy. A multidisciplinary approach can best determine whether acceptable outcomes can be expected and what management strategies may improve the prognosis for pregnant women with heart disease.

Long-term morbidity and quality of life after surgical repair of transposition of the great arteries: atrial versus arterial switch operation

Since a growing number of patients after surgical repair of transposition of the great arteries (TGA) survive until adulthood the focus of attention has shifted to the management of associated long-term morbidity and quality of life (QoL). Therefore, we reviewed all patients that underwent surgical repair of TGA at our institution and compared long-term results after atrial and arterial switch operation. Between 1973 and 2000, a total of 302 patients underwent either atrial switch operation (n=222) or arterial switch operation (n=80). Mean follow-up was 14.5±10.1 years. The arterial switch repair was associated with a higher early mortality whereas long-term survival was comparable between both groups. Postoperative arrhythmias including loss of sinus rhythm and pacemaker implantation occurred significantly more often after atrial switch repair. There was a trend towards a more favourable outcome of the arterial switch group concerning freedom from re-interventions, severe systemic ventricular dysfunction and need for heart failure medication. However, also the arterial switch operation was associated with an increased incidence of loss of sinus rhythm and neo-aortic valve regurgitation during late follow-up. Health related QoL according to the SF-36 questionnaire was not significantly different between both groups and comparable to a healthy population.

Should patients with congenital heart disease and a systemic ventricular ejection fraction less than 30% undergo prophylactic implantation of an ICD?

A number of important prospective studies have firmly validated the idea that patients at elevated risk for sudden cardiac death (SCD) can have that risk reduced by the use of implantable cardioverter defibrillators (ICDs). Among patients resuscitated from a potentially lethal cardiac event, ICD placement is termed secondary prevention. Primary prevention is the extension of this protective principle to groups of patients who have yet to experience a cardiac arrest, but who by virtue of specific and carefully defined clinical characteristics are determined to be at elevated risk for this event. Response by Silka and Bar-Cohen see p 317 Given the cost of ICD therapy and the number of patients who may meet primary prevention indications, the potential societal cost of this approach to cardiac risk management is high. In the past decade, this has driven a vigorous debate within the fields of heart rhythm management and health care economics as to the optimal ways to delineate patient groups that will most benefit from ICD therapy. The Multicenter Automatic Defibrillator Implantation Trial II (MADIT-II) study1 was a milestone in the evolution of this debate, providing evidence that a large group of patients—those with ischemic cardiomyopathy—could be rapidly and noninvasively sorted on the basis of left ventricular (LV) ejection fraction, with those having severe LV dysfunction realizing a clear survival benefit from ICD therapy. In this article, I challenge the proposition that MADIT-II and subsequent studies of the efficacy of ICD therapy in patients with acquired LV dysfunction can be applied to patients with moderate to severe systemic ventricular dysfunction in congenital heart disease (CHD). To make this argument, several topical areas must be considered. These include the current state of clinical knowledge of the natural history of CHD in adolescents and adults, the known rates of sudden death in …

Congenital heart disease in pregnancy.

Care of pregnant patients with congenital heart disease requires understanding of the specific congenital defect, the nature of previous surgical correction, and the residua and sequelae. General risks and principles can be adduced in management decisions. In addition, lesion- and patient-specific details are important. There are only a few conditions that place patients at a high enough risk to advise that pregnancy be avoided under all circumstances (pulmonary vascular obstructive disease, Marfan syndrome with dilated aortic root, severe aortic stenosis, and severe systemic ventricular dysfunction). Preconception counseling, optimization of status, and meticulous multidisciplinary management during pregnancy and the postpartum period will improve outcomes.