Lodewijk J. Wagenaar

Heart failure admissions in adults with congenital heart disease; risk factors and prognosis

BACKGROUND Heart failure (HF) is a serious complication and often the cause of death in adults with congenital heart disease (CHD). Therefore, our aims were to determine the frequency of HF-admissions, and to assess risk factors of first HF-admission and of mortality after first HF-admission in adults with CHD. METHODS The Dutch CONCOR registry was linked to the Hospital Discharge Registry and National Mortality Registry to obtain data on HF-admissions and mortality. Risk factors for both HF-admission and mortality were assessed using Cox regression models. RESULTS Of 10,808 adult patients (49% male), 274 (2.5%) were admitted for HF during a median follow-up period of 21 years. The incidence of first HF-admission was 1.2 per 1000 patient-years, but the incidence of HF itself will be higher. Main defect, multiple defects, and surgical interventions in childhood were identified as independent risk factors of HF-admission. Patients admitted for HF had a five-fold higher risk of mortality than patients not admitted (hazard ratio (HR)=5.3; 95% confidence interval 4.2-6.9). One- and three-year mortality after first HF-admission were 24% and 35% respectively. Independent risk factors for three-year mortality after first HF-admission were male gender, pacemaker implantation, admission duration, non-cardiac medication use and high serum creatinine. CONCLUSIONS The incidence of HF-admission in adults with CHD is 1.2 per 1000 patient-years. Mortality risk is substantially increased after HF-admission, which emphasises the importance to identify patients at high risk of HF-admission. These patients might benefit from closer follow-up and earlier medical interventions. The presented risk factors may facilitate surveillance.

Contemporary prevalence of pulmonary arterial hypertension in adult congenital heart disease following the updated clinical classification

BACKGROUND The aging congenital heart disease (CHD) population is prone to develop a variety of sequelae, including pulmonary arterial hypertension (PAH). Previous prevalence estimates are limited in applicability due to the use of tertiary centers, or database encoding only. We aimed to investigate the contemporary prevalence of PAH in adult CHD patients, using a nationwide population. METHODS A cross-sectional study was performed, using the population-based Dutch CONgenital CORvitia (CONCOR) registry. All patients born with a systemic-to-pulmonary shunt, thereby at risk of developing PAH, were identified. From this cohort, a random sample was obtained and carefully reviewed. RESULTS Of 12,624 registered adults with CHD alive in 2011, 5,487 (44%) were at risk of PAH. The random sample consisted of 1,814 patients (mean age 40 ± 15 years) and 135 PAH cases were observed. PAH prevalence in patients born with a systemic-to-pulmonary shunt was 7.4%. The prevalence of PAH after corrective cardiac surgery was remarkably high (5.7%). Furthermore, PAH prevalence increased with age, from 2.5% under 30 years until 35% in the eldest. PAH prevalence in the entire CHD population was 3.2%. Based on 3000 per million adult CHD patients in the general population, we can assume that PAH-CHD is present in 100 per million. CONCLUSIONS This new approach using a nationwide CHD population reports a PAH prevalence of 3.2% in CHD patients, and 100 per million in the general adult population. Especially in patients after shunt closure and the elderly, physicians should be aware of PAH-CHD, to provide optimal therapeutic and clinical care.

Three cases of hepatocellular carcinoma in Fontan patients: Review of the literature and suggestions for hepatic screening.

The Fontan procedure has been used since 1971 as a palliative treatment for various (functionally) univentricular hearts. The systemic venous blood flows passively to the pulmonary arteries, without passing through a functional ventricle. This results in chronic systemic venous congestion, which may lead to liver fibrosis, cirrhosis and hepatocellular carcinoma. This review discusses possible screening modalities for liver fibrosis and cirrhosis in the Fontan population and proposes a screening protocol. We suggest starting screening for progression of fibrosis and cirrhosis in collaboration with the hepatologist circa 10 years after Fontan completion. The screening programme will consist of a yearly evaluation of liver laboratory tests in conjunction with imaging of the liver with ultrasound or MRI every two years. In case of liver fibrosis or cirrhosis, (reversible) causes should be ruled out (e.g. obstruction in the Fontan circuit). In case of severe fibrosis or cirrhosis, other complications of portal hypertension should be evaluated and screening for hepatocellular carcinoma is required on a regular (6-12 months) basis. As regards hepatocellular carcinoma, treatment should be discussed in a multidisciplinary team, before deciding a treatment modality.

Pulmonary hypertension and pregnancy outcomes: data from the Registry Of Pregnancy and Cardiac Disease (ROPAC) of the European Society of Cardiology.

To describe the outcomes of pregnancy in women with pulmonary hypertension.

Percutaneous Tricuspid Valve Implantation in a Fontan Patient With Congestive Heart Failure and Protein-Losing Enteropathy

A 47-year-old patient visited our outpatient clinic. She was born with tricuspid atresia with normally connected great arteries without pulmonary stenosis (type Ic); a ventricular septal defect connected the pulmonary artery and hypoplastic right ventricle (RV) with the left ventricle. When she was 14 years old, a Fontan-Bjork procedure was performed.1 The atrial and the ventricular septal defects were closed and a valved conduit (Hancock prosthesis) was placed between the right atrium (RA) and the hypoplastic RV, thus providing pulsatile flow to the pulmonary arteries. Ten years later, the conduit was severely obstructed and replaced by a 23-mm aorta homograft. At the age of 38 years, she had atrioventricular block for which a dual-chamber pacemaker system was implanted with placement of the ventricular lead through the homograft in the RV apex. In the last 2 years, she had progressive heart failure and diarrhea. She was clinically diagnosed with protein-losing enteropathy (PLE), based on hypalbuminemia, edema, and diarrhea. Other …

Contrast-enhancement cardiac magnetic resonance imaging beyond the scope of viability

The clinical applications of cardiovascular magnetic resonance imaging with contrast enhancement are expanding. Besides the direct visualisation of viable and non-viable myocardium, this technique is increasingly used in a variety of cardiac disorders to determine the exact aetiology, guide proper treatment, and predict outcome and prognosis. In this review, we discuss the value of cardiovascular magnetic resonance imaging with contrast enhancement in a range of cardiac disorders, in which this technique may provide insights beyond the scope of myocardial viability.

Non-intubated recovery from refractory cardiogenic shock on percutaneous VA-extracorporeal membrane oxygenation

We report on the use of percutaneous femoral veno-arterial extracorporeal membrane oxygenation (VA-ECMO) in a fully awake, non-intubated and spontaneously breathing patient suffering from acute, severe and refractory cardiogenic shock due to a (sub)acute anterior myocardial infarction. Intensified heart failure therapy was closely monitored with a pulmonary artery catheter and allowed gradual weaning off the ECMO support without additional invasive measures, notably without mechanical ventilation. Neurological assessment was possible at all times and complete physical mobilisation was straightforward directly after weaning from ECMO. This limited invasive approach may encourage a more widespread use of percutaneous VA-ECMO.

Extensive Left Ventricular Hemangioma

![Figure][1] [![Graphic][3] ][3][![Graphic][4] ][4][![Graphic][5] ][5] A 27-year-old man with no medical history presented with ventricular tachycardia at 160 beats/min with right bundle branch block and left axis. Transthoracic echocardiography showed an echogenic mass

Relationship between Framingham Risk Score and Left Ventricular Remodeling after Successful Primary Percutaneous Coronary Intervention in Patients with First Myocardial Infarction and Single Vessel Disease

Background: Limited data is available on the potential value of estimated cardiovascular event risk for prediction of left ventricular (LV) remodeling and size of infarcted tissue after ST-elevation myocardial infarction (STEMI). Methods: Therefore, we assessed in a consecutive series of patients with first STEMI, successful primary percutaneous coronary intervention (PCI), and single-vessel disease the potential relationship between the Framingham Risk Score and parameters of both LV remodeling and infarct tissue characteristics, as determined with contrast-enhanced (CE) cardiovascular magnetic resonance (CMR) 6 months after the index event. Parameters of LV remodeling were end-diastolic and end-systolic volumes, ejection fraction, and wall motion score index; infarct tissue characteristics comprised core, peri, and total infarct size, and transmural extent. Results: A total of 25 patients (21 men, 56 ± 10 years) were studied, and the mean Framingham Risk Score was 14.1 ± 5.8%. There was a significant relation between Framingham Risk Score and multiple parameters of LV remodeling: LV ejection fraction, end-diastolic volume, end-systolic volume, and wall motion score index after 6 months (r=-0.55-0.76; p=0.000 for all). Framingham Risk Score showed no relation with various infarct tissue characteristics (ns). Male gender was the only component of the Framingham Risk Score that correlated individually with a few parameters of LV remodeling: LV end-diastolic volume and end-systolic volume (p=0.000 for both). Conclusion: In a series of consecutive patients with first STEMI, successful primary PCI, and single-vessel coronary artery disease, we observed a significant relation between the Framingham Risk Score and several CMRbased parameters of LV remodeling. The results of our small hypothesis-generating study underline the supremacy of multifactorial risk scores as tools for prediction of unfavorable cardiovascular outcome. Additionally, the data support the hypothesis that there might be a future role for a novel and specific multifactorial risk score in predicting unfavorable LV remodeling, which finally could trigger risk-adjusted preventive measures.

Obstructive intramyocardial haematoma after percutaneous coronary intervention

IntroductionNowadays, percutaneous coronary interventions are part ofroutine cardiology practice. However, serious complicationsmay occur (4% in severe coronary disease [ 1]). In this case, arare complication is described, where the use of on-siteimaging and surgery is underlined.CaseA 50-year-old man was referred to our centre to undergo anelectivepercutaneouscoronaryintervention(PCI).Thepatientwas admitted to the referring hospital with atrial fibrillationand a rise of his cardiac biomarkers. Coronary angiographywas performed showing a diffusely diseased right coronaryartery(RCA)withanelongatedstenosisinthemidsegmentof70%. Furthermore a 60% stenosis in the circumflex (RCX)and a chronic occluded obtuse marginal (OM) branch wereseen.Hismedicalhistoryincludedapriorposteriormyocardialinfarction 14 years ago treated by a PCI of his RCX.Medicationonadmissionincludedacetylsalicylicacid,sotalol,losartan, fluvastatin and ezetimibe.It was decided to perform a PCI of the RCA withplacement of drug-eluting stents. After passage of a guide-wire the lesion was directly stented with 3 Xience-V stents,dimensions 3.5×28 mm successively. After post-dilationuntil 30 atmosphere because of the massive calcification theangiographic result was satisfactory.Two hours later the patient was referred back for re-catheterisation because he developedthe clinicalsymptoms ofinferior myocardial infarction. He experienced chest pain,paleness, intense perspiration, bradycardia and hypotension.Electrocardiography showed ST-segment elevation in theinferior leads (Fig. 1). Repeat coronary angiography wasperformed showing extravasation of contrast (Fig. 2). Cardiacultrasound was carried out, showing a large intramyocardialmass in the right ventricular free wall, almost obliterating theright ventricular cavity (Fig. 3). Furthermore, a large amountof pericardial effusion, compressing the right side of theheart, was observed.Pericardiocentesis was performed, with evacuation of400 ml of blood. This did not improve his clinical status.Systolic blood pressure remained below 100 mmHg. Thecardiothoracic surgeon was consulted who subsequentlyperformed a surgical exploration through median sternotomy.A large amount of blood with thrombus was evacuated fromthe pericardium. Moreover, a large haematoma in the rightventricularfreewallhadrupturedinthepericardialspace.Theright ventricle was repaired with Teflon and Prolene, andsealed with Tacho-Seal and Tissue-col. The left internalmammary artery (LIMA) was distally anastomosed onthe OM branch. Patient was successfully weaned fromthe heart-lung machine. Due to poor haemodynamicstatus and decreasing haemoglobin rethoracotomy wasperformed 12 h later, with pinching off the side branchesof the LIMA and diathermic closure of several localarterioles.