Congenial Heart Disease at Adult Age

__Abstract__ Congenital cardiac defects are by far the most common congenital anomalies. Of all live births around the world, approximately 1% is born with congenital heart disease.1 This number is even higher if patients with a bicuspid aortic valve are included.2 Accordingly, in the Netherlands every year around 1800 children are born with a congenital heart defect. Of these, about 50% cure spontaneously or do well without medical or surgical treatment. In the 1960’s, before the development of cardiac surgery with cardiopulmonary bypass, about 50% of the children with congenital heart disease requiring therapy died within the first year of live and less than 15% reached adulthood.3 Especially patients with transposition of the great arteries had a very poor survival: 90% of the patients died in the first month of life and less than 1% reached adult age. Of tetralogy of Fallot patients 10% survived until adult age

Pregnancy in congenital heart disease: Risk prediction and counselling

Risk prediction and counselling are the key to limit risks of complications during pregnancy in women with congenital heart disease. The WHO classification and clinical risk tools will guide the physician to the best available risk estimate, but an individualised approach and expert opinion remains paramount in counselling women with congenital heart disease with a pregnancy wish. In women with an estimated low-risk or intermediate-risk pregnancy, planned follow-up and a delivery plan made by a multidisciplinary team provides the best chance of an uncomplicated pregnancy. While the majority do well, there is a small group of women that need an explicit advice not to embark pregnancy, to prevent devastating situations

Endovascular catheter ablation of ventricular tachycardia in a patient with a surgically repaired congenital left ventricular aneurysm

We present a patient with a congenital left ventricular aneurysm who visited our outpatient clinic for a routine check-up and, during this visit, lost consciousness due to sustained ventricular tachycardia. In our patient, endocardial mapping revealed extensive conduction abnormalities, and successful ablation was accomplished at the endocardial surface

Heart failure in congenital heart disease: management options and clinical challenges

Introduction: The population of adults with congenital heart disease (ACHD) is rapidly expanding and one of the major complications is heart failure. Timely diagnosis and treatment are crucial, but strong evidence for effectiveness of heart failure treatment in ACHD is currently lacking. Components of the medical history, physical examination and further diagnostic tests including ECG, echocardiography, cardiac magnetic resonance imaging, exercise testing, and biomarkers can identify patients at risk for early mortality or heart failure. Areas covered: Although the number of studies guiding evidence-based treatment are expanding, many clinical questions have not been completely answered yet. Therefore, in this review we provide an overview of current available insights in epidemiology, diagnosis, risk stratification and treatment options in ACHD patients, including non-medical therapies and advanced care planning. Expert Opinion: We strongly advocate expanding current use of biomarkers in the diagnostic process and timely initiation of discussing advanced treatment options and advanced care planning with patients and their loved ones. More research in multi-center collaborations is needed to study all aspects of care of adult congenital heart disease patients

Incidence and Predictors of Maternal Cardiovascular Mortality and Severe Morbidity in the Netherlands: A Prospective Cohort Study

Objective: To assess incidence and possible risk factors of severe maternal morbidity and mortality from cardiovascular disease in the Netherlands. Design: A prospective population based cohort study. Setting: All 98 maternity units in the Netherlands. Population: All women delivering in the Netherlands between August 2004 and August 2006 (n = 371,021) Methods: Cases of severe maternal morbidity and mortality from cardiovascular disease were prospectively collected during a two-year period in the Netherlands. Women with cardiovascular complications during pregnancy or postpartum who were admitted to the ward, intensive care or coronary care unit were included. Cardiovascular morbidity was defined as cardiomyopathy, valvular disease, ischaemic heart disease, arrhythmias or aortic dissection. All women delivering in the same period served as a reference cohort. Main outcome measures: Incidence, case fatality rates and possible risk factors. Results: Incidence of severe maternal morbidity due to cardiovascular disease was 2.3 per 10,000 deliveries (84/358,874). Maternal mortality rate from cardiovascular disease was 3.0 per 100,000 deliveries (11/358,874). Case fatality rate in women with severe maternal morbidity due to cardiovascular disease was 13% (11/84). Case fatality rate was highes

Reply: Letter to the editor: Prognostic value of left atrial size and function in adults with tetralogy of Fallot

_Dear Dr. An,_ We would like to thank you for your interest in our article entitled “Prognostic value of left atrial size and function in adults with tetralogy of Fallot”. We fully agree that accurate prognostication is of paramount importance in these patients.We are pleased to respond to the comments point by point. Indeed, other imaging modalities such as cardiac magnetic resonance (CMR) are probably more accurate than 2D echocardiography in the assessment of LA volumes. We have acknowledged this in our article in the Clinical Implications paragraph, in which we stated “Although other imaging techniques such as CT, CMR or 3D echocardiography could provide a more accurate estimation of LA size, (…)”. We also agree that the cohort of 32 patients that was analyzed in your department is probably too small to demonstrate any association between LA size and cardiovascular events. Future research is therefore certainly needed to evaluate the prognostic value of CMR-derived left atrial volumes in a large prospective cohort of patients with tetralogy of Fallot with a long-term follow-up duration. Nevertheless, the echocardiographic measurements that are investigated in our study can be easily implemented in day-to-day clinical practice and CMR also has its limitations such as higher costs, less availability, and inability to scan patients with intracardiac devices. As described in Tables 1, 67 patients (50%) had received a surgical PVR at the timeof study inclusion. In Table 2,we have described the cardiac function of all patients at the time of study inclusion. It was considered outside the scope of this study to describe cardiac function at the time of surgical PVR, because this was median 7.7 years prior to study inclusion. We hope that this additional information can adequately address the comments raised

Repetitive out of hospital cardiac arrests following pregnancy: A case report of an unfortunate presentation of mitral annular disjunction

Background Mitral annular disjunction (MAD) is an under-recognized cause of arrhythmic sudden cardiac death, especially in young women. The relation between MAD and the occurrence of arrhythmia during pregnancy has not yet been explored. We would like to stress the importance of careful echocardiographic examination and the vulnerable peripartum period. ................................................................................................................................................................................................... Case summary A 29-year-old woman survived an out of hospital cardiac arrest 4 months after delivery of her first child. The diagnosis was not clear and an implantable cardioverter-defibrillator (ICD) as secondary prevention was implanted. Her second pregnancy and delivery were uneventful. The 12-lead electrocardiogram demonstrated sinus rhythm with right bundle branch block, ventricular extra systoles (premature ventricular contractions), and a right superior axis, i.e. origin in the inferolateral basal left ventricle. Transthoracic 2D echocardiography showed myxomatous mitral valve disease with moderate mitral valve insufficiency with normal left and right heart dimensions and function. However, 4 weeks after delivery she experienced a sudden syncope at home. Implantable cardioverter-defibrillator reading revealed primary ventricular fibrillation, induced by a ventricular premature beat (VPB), terminated with a successful ICD shock. A frame-by-frame echocardiographic analysis of the mitral valve using biplane echocardiographic analysis allowed diagnosis of MAD with detachment of the root of the annulus from the posterolateral ventricular myocardium during systole. ................................................................................................................................................................................................... Conclusion Mitral annular disjunction is an under-recognized cause of arrhythmic sudden cardiac death. Biplane echocardiographic analysis of the mitral annulus can identify MAD and as such may help for risk stratification and sudden cardiac death prevention. Careful follow-up is necessary especially during pregnancy and the postpartum period

Managing cardiac emergencies in pregnancy

A multidisciplinary, high-risk team should evaluate all pregnant women presenting with an acute cardiac event. Such events are potentially devastating if not recognised early and treated by experienced specialists. Management decisions are determined by the severity of the maternal condition and fetal viability. The management of most cardiac complications is similar in pregnant and non-pregnant women. There are n

Systematic review and meta-analysis of music interventions in hypertension treatment: A quest for answers

Background: Adverse effects, treatment resistance and high costs associated with pharmacological treatment of hypertension have led to growing interest in non-pharmacological complementary therapies such as music interventions. This meta-analysis aims to provide an overview of reported evidence on the effica