Setting up an adult congenital heart programme

With the growing number of adult patients with congenital heart defects in the developing world, many institutions and practice groups are beginning or growing their adult congenital heart disease (ACHD) programmes. In this paper, we review:■ Practical considerations in establishing a clinic (Where will your patients come from? How many new referrals annually can you anticipate?);■ Transition and transfer challenges (can you avoid the loss to care of children with moderate and complex congenital heart defects? Is there an agreed age of transfer policy? Is there an available and effective transition process?);■ Institutional considerations (do you really have the support of your institution? Where should your program be located? Where will you admit your inpatients?);■ Patient care imperatives (how will you commit to excellent patient care? How will you meet the needs of your patients? Clearly designate which patients you will need to refer out and to which services they will be referred);■ The composition of the ACHD team (begin with two ACHD cardiologists and skilled echocardiography, consider a mix of pediatric trained and adult trained cardiologists, grow from there in a modular fashion if you can to include more multidisciplinary team members, and duplicate essentialservice positions); and■ Organisation and accreditation of ACHD care (ACHD services need to be managed differently than pediatric services across the full diagnostic and treatment clinicalspectrum)

Bleeding and thrombotic risk in pregnant women with Fontan physiology

Background/objectives Pregnancy may potentiate the inherent hypercoagulability of the Fontan circulation, thereby amplifying adverse events. This study sought to evaluate thrombosis and bleeding risk in pregnant women with a Fontan.  Methods We performed a retrospective observational cohort study across 13 international centres and recorded data on thrombotic and bleeding events, antithrombotic therapies and pre-pregnancy thrombotic risk factors.  Results We analysed 84 women with Fontan physiology undergoing 108 pregnancies, average gestation 33 +/- 5 weeks. The most common antithrombotic therapy in pregnancy was aspirin (ASA, 47 pregnancies (43.5%)). Heparin (unfractionated (UFH) or low molecular weight (LMWH)) was prescribed in 32 pregnancies (30%) and vitamin K antagonist (VKA) in 10 pregnancies (9%). Three pregnancies were complicated by thrombotic events (2.8%). Thirty-eight pregnancies (35%) were complicated by bleeding, of which 5 (13%) were severe. Most bleeds were obstetric, occurring antepartum (45%) and postpartum (42%). The use of therapeutic heparin (OR 15.6, 95% CI 1.88 to 129, p=0.006), VKA (OR 11.7, 95% CI 1.06 to 130, p=0.032) or any combination of anticoagulation medication (OR 13.0, 95% CI 1.13 to 150, p=0.032) were significantly associated with bleeding events, while ASA (OR 5.41, 95% CI 0.73 to 40.4, p=0.067) and prophylactic heparin were not (OR 4.68, 95% CI 0.488 to 44.9, p=0.096). Conclusions Current antithrombotic strategies appear effective at attenuating thrombotic risk in pregnant women with a Fontan. However, this comes with high (>30%) bleeding risk, of which 13% are life threatening. Achieving haemostatic balance is challenging in pregnant women with a Fontan, necessitating individualised risk-adjusted counselling and therapeutic approaches that are monitored during the course of pregnancy

Ventricular tachyarrhythmia during pregnancy in women with heart disease: Data from the ROPAC, a registry from the European Society of Cardiology

Objectives: To describe the incidence, onset, predictors and outcome of ventricular tachyarrhythmia (VTA) in pregnant women with heart disease. Background: VTA during pregnancy will cause maternal morbidity and even mortality and will have impact on fetal outcome. Insufficient data exist on the incidence and outcome of VTA in pregnancy. Methods and results: From January 2007 up to October 2013, 99 hospitals in 39 countries enrolled 2966 pregnancies in women with structural heart disease. Forty-two women (1.4%) developed clinically relevant VTA during pregnancy, which occurred mainly in the third trimester (48%). NYHA class > 1 before pregnancy was an independent predictor for VTA. Heart failure during pregnancy was more common in women with VTA than in women without VTA (24% vs. 12%, p = 0.03) and maternal mortality was respectively 2.4% and 0.3% (p = 0.15). More women with VTA delivered by Cesarean section than women without VTA (68% vs. 47%, p = 0.01). Neonatal death, preterm birth (< 37 weeks), low birthweight (< 2500 g) and Apgar score < 7 occurred more often in women with VTA (4.8% vs. 0.3%, p = 0.01; 36% vs. 16%, p = 0.001; 33% vs. 15%, p = 0.001 and 25% vs. 7.3%, p = 0.001, respectively). Conclusions: VTA occurred in 1.4% of pregnant women with cardiovascular disease, mainly in the third trimester, and was associated with heart failure during pregnancy. NYHA class before pregnancy was predictive. VTA during pregnancy had clear impact on fetal outcome

Direct measurement of porto-systemic gradient in a failing fontan circulation

We describe the case history of a 42-year-old man with cardiac cirrhosis, portal hypertension, and life-threatening variceal bleeding after Fontan revision surgery. Direct pressure measurements in the portal vein, though high, demonstrated only a modest portosystemic gradient (PSG), 9 mm Hg. A transjugular intrahepatic portosystemic shunt procedure was performed. This reduced the PSG (3 mm Hg). His bleeding was controlled. The patient's histopathological findings were identical to that previously documented in Fontan patients, raising the question of whether these subdiaphragmatic hemodynamics are representative of the broader failing Fontan population.<br/

Successful percutaneous ablation of ventricular tachycardia in congenitally corrected transposition of the great arteries, a case report

Ventricular tachycardia (VT) is rarely recognized in patients with congenitally corrected transposition of the great arteries (CCTGA). We describe a 48-year-old woman with CCTGA, systemic atrioventricular valve replacement for Ebsteinoid malformation of the valve, ventricular dysfunction and ventricular tachycardia related to the previous surgical scar. The patient had successful non-contact mapping and radiofrequency ablation of the offending tachycardia substrate