Coarctation of the Aorta: Diagnosis and Management.

Coarctation of the aorta (CoA) accounts for approximately 5-8% of all congenital heart defects. Depending on the severity of the CoA and the presence of associated cardiac lesions, the clinical presentation and age vary. Developments in diagnosis and management have improved outcomes in this patient population. Even after timely repair, it is important to regularly screen for hypertension. Patients with CoA require lifelong follow-up with a congenital heart disease specialist as these patients may develop recoarctation and complications at the repair site and remain at enhanced cardiovascular risk throughout their lifetime.

Maternal Factors in Pregnancy and Ethnicity Influence Childhood Adiposity, Cardiac Structure, and Function.

Importance: The links between maternal and offspring adiposity and metabolic status are well established. There is much less evidence for the impact of these relationships combined with ethnic background on cardiac structure and function in childhood. Objective: To test the hypothesis that ethnicity, maternal adiposity and glycemic status, and child adiposity affect cardiac structure and function. Design: A prospective cohort study. Setting: A single-center mother-child cohort study. The cohort is a subset of the international multi-center Hyperglycemia and Adverse Pregnancy Outcomes (HAPO) study. Participants: This study included 101 healthy pre-pubertal British-born children [56 White Europeans (WEs) and 45 South Asians (SAs)] with a median age of 9.1 years, range 6.0-12.2 years, at the time of the investigation. Main Outcomes and Measures: Anthropometric and echocardiographic measurements were made on the cohort. Maternal pregnancy and birth data were available. Relationships between maternal parameters (BMI and glucose status), child adiposity, and echo measures were assessed. Results: Despite no ethnic difference in BMI SDS at a median age of 9.1 years, SA children exhibited higher levels of body fat than WE children (whole body, right arm, and truncal fat all p < 0.001). SA children also exhibited greater changes in weight and height SDS but not BMI SDS from birth than WE children. As expected, maternal BMI correlated with child BMI (r = 0.28; p = 0.006), and body fat measures (e.g., whole body fat r = 0.25; p = 0.03). Maternal fasting glucose levels were associated with child body fat measures (r = 0.22-0.28; p = 0.02-0.05). Left ventricular (LV) indices were not different between SA and WE children, but E/A and E'/A' (measures of diastolic function) were lower in SA when compared with WE children. LV indices correlated positively to BMI SDS and body fat markers only in SA children. Maternal fasting and 2-h glucose were negatively correlated with E'/A' in SA children (r = -0.53, p = 0.015, and r = -0.49, p = 0.023, respectively) but not in WE children. Conclusion and Relevance: SA and WE children exhibit differences in adiposity and diastolic function at a median age of 9.1 years. Novel relationships between maternal glycemia, child adiposity, and cardiac structure and function, present only in SA children, were identified.

Cardiopulmonary exercise testing in the evaluation of liver disease in adults who have had the Fontan operation.

BACKGROUND: Liver disease (LD) is a long-term complication in patients with a single ventricle who have had the Fontan operation. A decline in cardiopulmonary exercise testing (CPET) variables is associated with increased risk of hospitalization, but its association with LD is unknown. AIM: To determine the association between CPET variables and LD in adults who have had the Fontan operation. METHODS: We retrospectively reviewed the medical records from two tertiary institutions. RESULTS: We identified 114 adults (≥18 years; mean 30.9±7.4 years) who had undergone the Fontan operation: 56% were women; 63% had total cavopulmonary connection; 66% had New York Heart Association (NYHA) class I status; 42% had arrhythmias; 22% had systemic right ventricle; and 35% had ventricular dysfunction. Of 81 patients with liver-imaging data, 41% had LD (i.e. imaging evidence of cirrhosis, with or without portal hypertension, splenomegaly or varices). There were no differences in clinical or echocardiographic variables between those with and without LD. Among the 58 patients with CPET data, mean peak oxygen consumption (VO2) was 18.6±5.7mL/kg/min, per-cent-predicted peak VO2 was 53.9±15.5%, peak oxygen pulse was 9.3±2.9mL/beat and per-cent-predicted peak oxygen pulse was 82.6±21.5%. Of the 44 patients with liver and CPET data, each standard deviation decrease in per-cent-predicted peak VO2 (16%) and per-cent-predicted peak oxygen pulse (22%) was associated with a 2.3-fold increase in the odds of LD, after adjusting for NYHA, institution and Fontan type (P=0.04). Similarly, each standard deviation decrease in per-cent-predicted peak VO2 and oxygen pulse was associated with an estimated 5.9-year and 4.9-year earlier onset of LD, respectively (P>0.05). CONCLUSIONS: Decline in per-cent-predicted peak VO2 and oxygen pulse was associated with increased odds of LD in adults who had undergone the Fontan operation. Our study supports more rapid hepatic evaluation among patients with abnormal or worsening CPET variables.

Systolic Anterior Motion Obstructing the Pulmonary Outflow Tract After Tricuspid Valve Replacement.

A patient with congenitally corrected transposition of the great arteries who presented with shortness of breath was found to have severe tricuspid regurgitation and right ventricular impairment. After uneventful mechanical systemic tricuspid atrioventricular valve replacement, the patient was extubated within 12 hours. On the first postoperative day, he developed episodes of profound hypotension lasting a few seconds. A transesophageal echocardiogram demonstrated displacement of the interventricular septum that caused systolic anterior motion of the mitral valve into the subpulmonic left ventricle. Mitral valve replacement resolved this complication, and the patient proceeded to do well at discharge.

Benefit of endovascular stenting for aortic coarctation on systemic hypertension in adults.

BACKGROUND: Endovascular stenting is a recognised treatment strategy for aortic coarctation (CoA) in adults. We assessed systemic hypertension control and the need for antihypertensive therapy after CoA stenting in adults. METHODS: Data were collected prospectively on 54 patients (36 men; mean age: 34 ± 16 years) who underwent endovascular stenting for CoA over a 7-year period. Five patients were excluded as they did not attend follow-up appointments. Patients underwent clinical examination, including right arm systolic blood pressure (SBP) and 24-hour ambulatory blood pressure monitoring at baseline, 6-12 weeks and 9-12 months. RESULTS: There was a significant fall in mean peak-to-peak systolic gradient (PG) across the CoA after stenting (26 ± 11 mmHg vs. 5 ± 4 mmHg; P<0.01). There were successive reductions in right arm SBP and ambulatory SBP at baseline, 6-12 weeks and 9-12 months post-procedure (right arm: 155 ± 18 mmHg vs. 137 ± 17 mmHg vs. 142 ± 16 mmHg, respectively; all P-values <0.01; ambulatory: 142 ± 14 mmHg vs. 132 ± 16 mmHg vs. 131 ± 15 mmHg, respectively; all P-values <0.01). Twenty-four patients had severe CoA (PG >25 mmHg before stenting); baseline SBP was significantly higher in severe versus non-severe patients (160 mmHg vs. 148 mmHg; P=0.02). The absolute reduction in PG after stenting was significantly higher in the severe group (31 ± 7 mmHg vs. 14 ± 5 mmHg; P<0.0001), but there was no significant difference in SBP between groups at 6-12 weeks (141 mmHg vs. 135 mmHg; P=0.21) or 9-12 months (139 mmHg vs. 139 mmHg; P=0.96). CONCLUSION: Endovascular stenting of CoA results in a significant reduction in SBP at 6-12 weeks, which is sustained at 9-12 months, with similar outcomes in severe and non-severe CoA groups.

Right and left ventricular strain and strain rate in young adults before and after percutaneous atrial septal defect closure.

To evaluate acute change of right and left ventricle after percutaneous closure of isolated atrial septal defect (ASD) 21 adult patients (13 F; 8 M) aged 28 ± 9.5 range 18-49 years have been examined by echocardiography before and 24 hours after percutaneous closure of ASD. Twenty-one normal adult subjects, as control group were included. A MyLab25 echo machine equipped with a multifrequency 2.5-3.5 MHz transducer was used. Offline computer-based analysis for strain and SR were performed using XStrain software based on a feature tracking algorithm. All patients had ASD OS2 with right ventricular dilatation and diastolic areas were larger than in controls: P = 0.0158. Global right ventricular longitudinal strain was higher P = 0.0438. Twenty-four hours after ASD closure, right ventricular diastolic and systolic areas were significantly reduced. Right ventricular global longitudinal systolic strain decreased: P = 0.00016, as well as global right ventricular longitudinal SR -1.56/sec ± 0.57 vs. -1.28/sec ± 0.31, P = 0.02646. At the mean time left ventricular end diastolic volume and left ventricular cardiac output measured by two-dimensional echocardiography both increased significantly P = 0.002145 and 0.013409. Global circumferential strain at mitral level augmented significantly -20.3%± 4.64 vs. -25.39%± 5.22, P = 0.00003. Longitudinal strain of the right ventricle works as indicator of right ventricular function dependent on loading conditions while SR seems to be less dependent on it. Circumferential strain could be used as an indicator of left ventricular response to normalized loading conditions.

Transcatheter closure of postsurgical residual ventricular septal defects: early and mid-term results.

BACKGROUND: The incidence of residual ventricular septal defects (VSDs) after surgery is 5-25%. Redo surgery is associated with higher risks. METHODS: Between January 2000 to December 2008, 170 patients underwent percutaneous VSD closure in our centre: 22(16M) of these had 23 closures for residual VSDs. Median age was 32.5 yrs (1.4-79). All patients had echocardiographic signs of left ventricle volume overload (Q(p)/Q(s) >or= 1.5). Nine patients had previous VSD closure, 6 tetralogy of Fallot repair, and 7, other procedures. There were 15 muscular, 6 perimembranous and 2 apical VSDs. RESULTS: Amplatzer VSD devices were used in all. Median VSD size was 8 mm (4.3-16). Median fluoroscopy time was 33 minutes (15-130). There were three adverse events: 1 ventricular fibrillation requiring DC cardioversion; 1 transient complete atrio-ventricular block reverting to sinus rhythm at 24-hours; one patient had transient atrial flutter during the procedure. All procedures were successful; no additional procedures were required. Trivial residual shunts were seen in 3 patients at follow-up. There were no late events. One patient experienced arrhythmic death 5-yrs after procedure. One patient was reoperated due to dehiscence of VSD patch 2-yrs after the second successful percutaneous closure. CONCLUSIONS: Transcatheter closure of postsurgical residual VSD is safe and efficacious management option and obviates the need for further surgery and by-pass.

Exercise training in adults with congenital heart disease: feasibility and benefits.

BACKGROUND: Regular physical activity is associated with a range of health benefits; however the influence of regular exercise training on exercise capacity and quality of life in adults with congenital heart disease [ACHD] has not been previously reported. METHODS: Prospective study of patients attending the ACHD clinic in a supra-regional centre, assessing feasibility and effects of exercise training. There were 3 phases: Phase I: Initial assessment including quality of life and physical activity questionnaires; treadmill exercise test; physical activity assessment with accelerometers [Caltrac and Actigraph]; Phase II: Exercise training (home-based walking 5/7 days) for 10 weeks; Phase III: Re-assessment. RESULTS: Sixty-one adults [36 males; mean age 31.7+/-10.9 yrs] were divided into 3 groups according to NYHA class. Fifty patients completed the intervention and all pre-post assessments. Group I (n=21; 13 males), Group II (n=16; 10 males), Group III (n=13, 10 males). Median body mass index was 23.8; 12 were overweight (BMI 25-29.9) and 7 obese (BMI>30). The scores of the quality of life and physical activity questionnaires increased significantly after exercise training in all three groups. Similar increases were seen in the treadmill test duration and in physical activity levels assessed by the Caltrac and the Actigraph in all groups. CONCLUSIONS: A simple physical activity intervention like regular walking is feasible, safe and significantly increases the exercise capacity of adult patients at all stages of congenital heart disease. It is also helpful in improving the quality of life by improving physical self-perception, satisfaction with life, physical activity levels and general health.

Initial experience with the new Amplatzer Duct Occluder II.

BACKGROUND: Different devices are used for transcatheter occlusion of patent ductus arteriosus (PDA), each with its own limitations and complications. We report our initial single-center experience with the new Amplatzer Duct Occluder II (ADO II), which has been designed to address some of these issues. METHODS: From April until October 2008, 10 consecutive patients with PDA (minimal diameter > or = 2 mm) were enrolled for intention-to-treat with ADO II. The median age was 10.2 years (7 months to 51.4 years); the median weight was 35.5 kg (6.3-70 kg). RESULTS: ADO II implantation was attempted in 10/10 patients. The mean PDA diameter was 3.1 mm (+/- 1.0 mm), and the mean device waist diameter was 4.0 mm (+/- 1.3 mm). In 1 patient, the ADO II encroached upon the left pulmonary artery, hence it was changed to an Amplatzer Duct Occluder I. In another patient, the shape and size of the duct necessitated the use of a Cook detachable coil. Complete angiographic closure was achieved in 8/8 ADO II implantations (100%) and in the patient with the coil. In the patient with an ADO I, complete echocardiographic closure was demonstrated at 24 hours. No device embolization or complications related to implantation occurred. CONCLUSIONS: This initial experience demonstrates the feasibility and efficacy of the ADO II to occlude PDA with a minimum diameter > or = 2 mm. It is especially useful in smaller children with larger duct diameters. Longer-term follow up in a larger cohort of patients is required to establish safety and long-term results.

Physical activity levels in adults with congenital heart disease.

BACKGROUND: Regular physical activity is associated with many health benefits in patients with cardiac disease. Advances in treatment have resulted in an increasing population of adults with congenital heart disease. Few data are available on physical activity levels and attitude to exercise in this group. DESIGN: Prospective, cross-sectional study of patients attending the adult congenital heart disease clinic in a supra-regional centre. METHODS: Physical activity assessed over 1 week using two accelerometers. The Actigraph was used to measure total activity volume and intensity and the Caltrac used to measure energy expenditure in physical activity. Patients completed a questionnaire to assess exercise self-efficacy. RESULTS: In all, 61 adults recruited (36 male; mean-age 31.7+/-10.9 years); divided into three groups according to New York Heart Association class [Group I (n=26; 14 male), Group II (n=18; 10 male), Group III (n=17; 11 male)]. Fourteen patients were overweight (body mass index 25-29.9) and seven obese (body mass index>30). Group 1 achieved similar activity levels to a sedentary reference population. Group II and III had significantly lower activity (55 and 40% of Group I). Most study patients failed to achieve UK national guidelines for physical activity (Groups I: 77%, II: 84%, III: 100%). Despite this, questionnaire responses suggested a willingness to participate in exercise in the majority of each group. CONCLUSIONS: Adults with congenital heart disease have a range of physical activity levels between normal and severely limited. Most patients showed a willingness to participate in exercise but were uncertain of the safety or benefit. Intervention to increase physical activity levels may be a low risk, low cost treatment strategy.

Axillary artery approach for balloon valvoplasty in young infants with severe aortic valve stenosis: medium-term results.

OBJECTIVES: To assess the feasibility and results of trans-axillary approach for balloon aortic valvoplasty (BAV) in early infancy. BACKGROUND: Severe aortic valve stenosis (SAVS) is rare but serious condition in infancy, which may be promptly treated either by surgical aortic valvotomy or BAV. BAV is usually performed via the femoral artery route, which is associated with significant vascular complications and long procedure times. METHODS: BAV via the trans-axillary approach was performed on twenty-seven sequential infants with SAVS presenting to a single tertiary referral center over an 11-year period. Maximum inflated balloon size was less than or equal to the aortic valve diameter. RESULTS: Twenty-seven infants aged 1-77 days underwent BAV. Weight at time of procedure was 2.0-4.42 kgs. The median procedure and screening times were 82 and 7.9 minutes, respectively. Mean instantaneous Doppler gradient across the aortic valve reduced from 68 +/- 33 to 37 +/- 14 mmHg ( p < 0.0001). Three infants developed at least moderate aortic regurgitation. Right arm pulse volume was decreased in 12 infants; 5 received an intravenous heparin infusion. Longer-term follow-up demonstrated reduced or absent peripheral pulse in 5 infants. Transection of the axillary artery occurred in one infant requiring emergency microvascular repair. There was one post-procedural and one late death due to non-cardiac causes. CONCLUSIONS: In early infancy balloon aortic valvoplasty via the axillary artery approach for severe aortic stenosis is an acceptable and safe alternative to the femoral arterial approach and results in short procedure and screening times. Longer-term vascular follow-up is required. (c) 2006 Wiley-Liss, Inc.