Pre-pregnancy counseling for women with heart disease: A prospective study.

BACKGROUND: Women with cardiac disease and their infants are at a greater risk of mortality and morbidity during pregnancy. Expert groups recommend preconception counseling (PCC) for all women with cardiac disease so they are made aware of these risks. We have run a specialist maternal cardiac clinic since 1996. The aim of this study was to evaluate the experience of women who have received PCC within an established multidisciplinary tertiary clinic and to establish their views regarding the counseling they received. METHODS: Single centre prospective study using a patient questionnaire was given to women attending a specialist cardiac preconception counseling clinic from November 2015 to August 2016, with analysis of descriptive data and free text comments from the questionnaire responders. RESULTS: 40/65 returned patient questionnaires. Prior to the consultation fewer than half felt well informed regarding how their heart disease could impact upon pregnancy but a similar proportion felt nonetheless that they would be able to have a healthy pregnancy. Women reported two main areas of concerns, their own health (whether they would survive a pregnancy) and the health of their child. 15% of women reported that these concerns had prevented them from pursuing a pregnancy. Women reported high satisfaction rates with the clinic. CONCLUSIONS: There is an increasing demand for PCC services for women with cardiac disease; our study is the first attempt to determine both the acceptability and the impact of PCC from the patient perspective. Patients reported a high level of satisfaction with the service provided.

Ventricular function and ventriculo-arterial coupling after palliation of hypoplastic left heart syndrome: A comparative study with Fontan patients with LV morphology.

BACKGROUND: Conceptually the right ventricle (RV) is less suitable to support the Fontan circulation than the left (LV). After palliation of hypoplastic left heart syndrome (HLHS) involving aortic reconstruction during the Norwood procedure the RV is exposed to abnormal afterload. We studied ventricular function and ventriculo-arterial coupling in HLHS patients (RV) and Fontan patients with single LV morphology that did (LV+N) and did not (LV-N) undergo Norwood-type aortic reconstruction. METHODS: Eighty patients (55 RV, 8 LV+N, 17 LV- N) were simultaneously studied with the conductance-catheter and echocardiography 4.8 (0.9-22.9)years after Fontan completion. RESULTS: Ejection fraction (EF) was lowest in the HLHS group (RV 60.9±11.0 vs. LV+N 68.4±10.5 vs. LV-N 69.7±8.0, P=0.003) whereas end systolic elastance (Ees), i.e. ventricular contractility, and end diastolic stiffness (Eed) were highest (Ees: RV 3.38±2.2 vs. LV+N 2.3.±13.8 vs. LV-N 1.92±1.37mmHg/ml, P=0.02; Eed: RV 0.59±0.36 vs. LV+N 0.48±0.29 vs. LV-N 0.32±0.17mmHg/ml, P<0.02). Arterial elastance, a measure of afterload, was highest in HLHS patients and correlated positively with Ees and Eed and inversely with EF in the study cohort. Only long axis function analysis suggested superior ventricular function in HLHS patients whereas all other echocardiographic measures did not reveal any group differences. CONCLUSION: Ventricular contractility of the RV of HLHS patients is higher than that of the ventricle of Fontan patients with LV morphology. This likely reflects a physiological response to higher arterial elastance resulting from aortic arch reconstruction. Increased arterial elastance negatively impacts diastolic stiffness, which is higher in the systemic RV than LV.

Prevalence and prognostic implication of restenosis or dilatation at the aortic coarctation repair site assessed by cardiovascular MRI in adult patients late after coarctation repair.

BACKGROUND: Cardiovascular magnetic resonance (CMR) is ideal for assessing patients with repaired aortic coarctation (CoA). Little is known on the relation between long-term complications of CoA repair as assessed by CMR and clinical outcome. We examined the prevalence of restenosis and dilatation at the repair site and the long-term outcome in patients with repaired CoA. METHODS AND RESULTS: CMR imaging and clinical data for adult CoA patients (247 patients aged 33.0 ± 12.8 years, 60% male), were analyzed. The diameter of the aorta at the repair site was measured on CMR and its ratio to the aortic diameter at the diaphragm (repair site-diaphragm ratio, RDR) was calculated. Restenosis (RDR≤70%) was present in 31% of patients (and significant in 9% [RDR<50%]), and dilatation (RDR>150%) in 13.0%. A discrete aneurysm at the repair site was observed in 9%. Restenosis was more likely after resection and end-end anastomosis, whereas dilatation after patch repair. Systemic hypertension was present in 69% of patients. Of the hypertensive patients, blood pressure (133 ± 20/73 ± 10 mm Hg) was well controlled in 93% with antihypertensive therapy. Mortality rate over a median length of 5.9 years was low (0.69% per year, 95% CI: 0.33-1.26), but significantly higher than age-matched healthy controls (standardised mortality ratio 2.86, CI 1.43-5.72, p<0.001). CONCLUSION: Restenosis or dilatation at the CoA repair site as assessed by CMR is not uncommon. Medium term survival remains good, however, albeit lower than in the general population. Life-long follow-up and optimal blood pressure control are likely to secure a good longer term outlook in these patients.

Experience with transcatheter closure of secundum atrial septal defects using the Amplatzer septal occluder: a single centre study in 236 consecutive patients.

AIM: To evaluate the safety and efficacy of transcatheter closure of secundum atrial septal defects (ASD) with the Amplatzer septal occluder. METHODS: 236 consecutive patients with a significant ASD (age 6 months to 46 years, median 5 years; body weight 6.5-79 kg, median 18 kg) were considered for transcatheter closure with the Amplatzer septal occluder; 18 patients with defects that were too large or with a deficient inferior margin were excluded from attempted transcatheter closure after initial transthoracic (4) or transoesophageal echocardiography (14). RESULTS: At cardiac catheterisation, devices were not implanted in 18 patients because the stretched diameter of the ASD was too large (4), the device was unstable (4), compromised the mitral valve (1), or obstructed the upper right pulmonary vein (1); eight patients with additional systemic or pulmonary vein anomalies (5) or a Qp:Qs less than 1.5 (3) were excluded after angiographic and haemodynamic assessment. Thus ASD closure was done successfully in 200 patients (procedure time 25-210 minutes, median 66 minutes; fluoroscopy time 2.5-60 minutes, median 12 minutes), among whom 22 had multiple ASDs (14) or a septal aneurysm (8). The diameter of the devices ranged between 6-34 mm. Severe procedure related complications (retroperitoneal bleeding, air embolism) occurred in two cases. At follow up (33 days to 4.3 years, median 2.3 years) complete closure was documented in 94%, with a trivial residual shunt in 12 patients. CONCLUSIONS: The Amplatzer septal occluder is very efficient and offered interventional ASD closure in 84.7% of our group of consecutive patients, with excellent intermediate results.

Influence of the pulmonary annulus diameter on pulmonary regurgitation and right ventricular pressure load after repair of tetralogy of Fallot.

OBJECTIVE: To assess the influence of the pulmonary annulus diameter after reconstruction of the right ventricular (RV) outflow tract at repair of tetralogy of Fallot on pulmonary regurgitation and RV pressure load; and to evaluate the impact of pulmonary regurgitation on RV size and function. SETTING: Paediatric cardiology and diagnostic radiology departments of a tertiary referral centre. PATIENTS: 67 patients were examined at a median of 4.8 years after repair of tetralogy of Fallot by means of biplane angiocardiography and magnetic resonance imaging (MRI). MAIN OUTCOME MEASURES: Pulmonary annulus diameter and area, pulmonary regurgitant fraction, RV to left ventricular (LV) systolic pressure ratio, RV end diastolic volume, and RV ejection fraction were assessed. RESULTS: There was a significant positive correlation between pulmonary annulus area indexed to body surface area and pulmonary regurgitation (angiocardiography: r = 0.55, p < 0.001; MRI: r = 0.59, p < 0.001). No significant correlation was found between pulmonary annulus index and RV to LV systolic pressure ratio even in patients with small pulmonary annulus areas (r = -0.24, NS). Pulmonary regurgitant fraction was positively correlated with normalised RV end diastolic volume (angiocardiography: r = 0.42, p < 0.05; MRI: r = 0.56, p < 0.01). RV ejection fraction decreased with increasing pulmonary regurgitation (angiocardiography: r = -0.42, p < 0.05; MRI: r = -0.41, p < 0.05). CONCLUSIONS: The extent of pulmonary regurgitation after tetralogy of Fallot repair correlates with the postoperative size of the pulmonary annulus and is closely correlated with the enlargement of the RV. An enlargement of the pulmonary annulus to the second lower standard deviation of normal results in a decrease of pulmonary regurgitation and is sufficient to achieve adequate RV pressure unloading.

Transcatheter closure of persistent ductus arteriosus in infants using the Amplatzer duct occluder.

AIM: To evaluate whether transcatheter closure with the Amplatzer duct occluder offers an alternative to surgical treatment in infants with a persistent ductus arteriosus. METHODS: 12 patients under 1 year of age (age 1-11 months, body weight 2.6-8.7 kg) with clinical and echocardiographic findings of a significant duct were considered for transcatheter closure with the Amplatzer occluder. The device is made of a Nitinol and polyester fabric mesh and provides occlusion by stenting the duct. Measured angiographically, the narrowest diameter of the ducts ranged from 1.5-5 mm; in six patients pulmonary hypertension was also present. RESULTS: The devices were implanted and complete duct occlusion was demonstrated during follow up in 10 patients. Procedure related difficulties occurred in nine of the 12 cases and led to relatively long procedure and fluoroscopy times (procedure time 50-180 minutes, median 80 minutes; fluoroscopy time 4.9-49 minutes, median 16 minutes). In two infants transcatheter closure could not be achieved and surgical duct ligation had to be carried out. CONCLUSIONS: In small infants with a persistent ductus arteriosus the Amplatzer duct occluder offers an alternative to surgical treatment, but further improvement of the implantation system is necessary before the procedure can be recommended as the treatment of choice.

[Atrial ectopic tachycardia in infancy caused by tumor of the interatrial septum]. / Atriale ektope Tachykardie im Säuglingsalter durch Tumor des interatrialen Septums.

A case of atrial ectopic tachycardia in an infant probably caused by a small tumor in the interatrial septum is presented. The arrhythmia was successfully treated with amiodarone. Several histologic abnormalities are known to cause AET, but an intracardiac tumor detectable by two-dimensional echocardiography is extremely rare. AET -especially in childhood-as well as cardiac tumors show a tendency to regression, so that even in this case medical treatment should be the first therapeutic choice. Interventional or surgical treatment is required just for those patients with AET this is resistant to medical treatment or reoccurs after finishing the medical treatment.

Intracoronary magnesium is not protective against acute reperfusion injury in the regional ischaemic-reperfused dog heart.

Intravenous magnesium lowers mortality in patients with suspected myocardial infarction. We tested the hypothesis that the protective effect may be due to a direct, local influence of magnesium on myocardial reperfusion injury in a dog model of ischaemia/reperfusion. Ten anaesthetized open chest dogs underwent 1 h of left anterior descending artery (LAD) occlusion and 6 h of reperfusion. The animals received intracoronary (i.c.) magnesium aspartate (Mg, n = 5) or vehicle infusion (n = 5) for the first hour of reperfusion. Mg infusion was adapted to actual LAD flow (ultrasonic flow probe) to increase regional plasma concentration by 4 mmol L-1. Regional myocardial function was measured as percent systolic wall thickening (sWTh, sonomicrometry). Intracoronary Mg increased LAD flow during application (at 15 min reperfusion; Mg, 194 +/- 44 (mean +/- SD); control, 116 +/- 41 mL min-1 100 g-1, P < 0.01). sWTh decreased during coronary occlusion from 14.3 +/- 7.1% to -4.7 +/- 2.7% in the control group and from 14.8 +/- 2.5% to -4.1 +/- 3.1% in the Mg group. Throughout the reperfusion period wall function remained depressed in both groups to a similar degree (control, -3.5 +/- 1.8%; Mg, -3.0 +/- 1.9% at 6 h reperfusion). Global haemodynamics were not different. Infarct size after 6 h reperfusion (TTC staining) was similar in both groups (Mg, 20.6 +/- 5.0; control, 24.4 +/- 8.7% of area at risk). Regional magnesium application (i.c.) to post-ischaemic reperfused myocardium had no influence on infarct size or post-ischaemic regional wall function in this model.(ABSTRACT TRUNCATED AT 250 WORDS)

Intracoronary SIN-1C during reperfusion reduces infarct size in dog.

Reperfusion of ischemic myocardium may aggravate the ischemic state of injury and thus augment infarct size (reperfusion injury). The aim of this study was to reduce infarct size by an intervention at the time of reperfusion that acts only on a reperfusion-specific pathomechanism. It was investigated whether SIN-1C, a metabolite of molsidomine, can protect against reperfusion injury in canine hearts in vivo. Ten anesthetized open chest dogs underwent 1 h of left anterior descendent artery (LAD) occlusion and were randomly assigned to receive either intracoronary SIN-1C or vehicle infusion as a placebo during the first hour of reperfusion. The infusion was adjusted to LAD flow to achieve a regional blood concentration of 5 x 10(-3) M. Infarct size was assessed by triphenyltetrazolium staining after 6 h of reperfusion. Left ventricular pressure (LVP) was similar in both groups (SIN-1C: 101 +/- 6, placebo: 89 +/- 6 mm Hg, mean +/- SEM, n = 5) at the beginning of the experiment and did not change significantly thereafter from baseline values in both groups. During SIN-1C infusion, the LAD flow was increased (SIN-1C: 195 +/- 38, control: 86 +/- 17 ml/min/100 g at 30 min of reperfusion, p < 0.05), while systemic hemodynamics remained unaltered. A reduction in infarct size (percent of area at risk) was seen in the SIN-1C group (11.4 +/- 2.8%) compared with the placebo group (24.4 +/- 3.9%, p < 0.05). Infusion of papaverin (5 x 10(-5) M) following an identical protocol caused a similar vasodilation as SIN-IC, but did not reduce infarct size in five additional dox experiments.(ABSTRACT TRUNCATED AT 250 WORDS)

Regional contractile blockade at the onset of reperfusion reduces infarct size in the dog heart.

An important mechanism of lethal myocardial reperfusion injury is the development of cellular hypercontracture at the onset of reperfusion. Hypercontracture can lead to cytolysis by mutual mechanical disruption of myocardial cells. 2,3-Butanedione monoxime (BDM) inhibits myofibrillar cross-bridge cycling and may therefore reduce infarct size in ischaemic reperfused myocardium. This study investigated whether a temporary presence of BDM protects against myocardial reperfusion injury in an intact-animal preparation. Anaesthetized open-chest dogs (n = 10) underwent 1 h of left anterior descendent artery (LAD) occlusion and received intracoronary BDM (25 mM, n = 5) or vehicle (n = 5) for 65 min starting with an anoxic local infusion 5 min before reperfusion. Infarct size was assessed by triphenyltetrazolium staining after 6 h reperfusion. The infusion of BDM was accompanied by a transient reduction of left ventricular systolic pressure from 84.3 +/- 11.2 mm Hg during occlusion to 66.4 +/- 9.9 mm Hg at 30 min reperfusion (mean +/- SD, P < 0.01 vs. control). LAD-flow and regional wall motion in the area at risk showed no difference between groups. Infarct size (% of area at risk) was reduced from 24.4 +/- 8.7 (control) to 6.6 +/- 2.0% (BDM) (P < 0.01). The results demonstrate that development of necrosis in reperfused myocardium can be greatly reduced by temporary presence of the contractile inhibitor BDM at the onset of reperfusion.

Adenosine A2-receptor activation at reperfusion reduces infarct size and improves myocardial wall function in dog heart.

Reestablishment of blood supply to ischemic myocardium leads to biochemical and cellular changes which are believed to reduce the amount of potentially salvageable myocardium (reperfusion injury). In this situation, adenosine is known to have myocardial protective properties. Activation of adenosine A2-receptors may account for most of the beneficial effects of adenosine in reperfusion injury because A2-receptor activation mediates vasodilation, inhibits neutrophil adhesion to vascular endothelium and diminishes generation of free radicals by neutrophils, thus acting on some of the key mechanisms of reperfusion injury such as postischemic vascular dysfunction and neutrophil-mediated damage. Therefore, we investigated the effect of an intracoronary A2-agonist, CGS 21680, on regional postischemic myocardial function (measured as wall thickening) and infarct size [determined by triphenyltetrazolium chloride (TTC) staining]. Fourteen anesthetized open-chest dogs underwent 1-h left anterior descending artery (LAD) occlusion and 6-h reperfusion and were randomly assigned to receive intracoronary CGS 21680 or to serve as control. The drug was infused for 60 min starting 5 min before reperfusion with a concentration of 10(-7) M at a rate of 10 ml/min under anoxic conditions. The infusion was then continued for the first 55 min of reperfusion with 10(-6) M at a rate of 1 ml/min. Intracoronary infusion of CGS 21680 led to significant improvement in regional wall function in postischemic myocardium (p < 0.05 vs. control). Thickening fraction (percentage of baseline) increased from -13.1 +/- 13.7% (mean +/- SD) during occlusion to 15.3 +/- 29.8% at 30 min of reperfusion in the CGS 21680 treatment group and remained at this level throughout the reperfusion period.(ABSTRACT TRUNCATED AT 250 WORDS)