Congenital heart disease combined with Arrhythmogenic Right Ventricular Cardiomyopathy: A CARE compliant case report and literature review.

RATIONALE: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a hereditary cardiomyopathy disease discovered in 1994. Though there are advances in diagnosis of arrhythmogenic right ventricular cardiomyopathy, early diagnosis is still difficult especially when it is combined with other diseases with similar pathophysiologic changes, such as left to right shunt congenital heart disease (CHD). In this paper, we reported a case of CHD combined with ARVC. PATIENT CONCERNS: The patient was referred to us for chest tightness and shortness of breath after physical activities. His cardiac MRI indicated the possibility of arrhythmogenic right ventricular cardiomyopathy. He was diagnosed with a large atrial septal defect (ASD) through ultrasound examination. DIAGNOSIS: CHD ASD combined with arrhythmogenic right ventricular cardiomyopathy. INTERVENTIONS: The patient underwent occlusion of the ASD and he was followed-up closely. His symptoms were relieved a lot and the activity tolerance was elevated. LESSONS: CHD may accompany with arrhythmogenic right ventricular cardiomyopathy. Careful history collection and comprehensive examinations should be emphasized. We firmly believe that our work will be helpful for the medical practice of similar complicated cardiovascular diseases.

Effect of complex cardiac rehabilitation on physical activity and quality of life during long-term follow-up after surgical correction of congenital heart disease.

BACKGROUND: Comprehensive cardiac rehabilitation (CCR) is an important element of the comprehensive management of grown-up congenital heart disease (GUCH) patients after surgical correction of congenital heart disease (CHD) but access to this treatment is still limited. We still lack Polish guidelines on CCR, including controlled training in young adults several years after surgical correction of CHD. AIM: To assess the effect of a CCR program on physical capacity, exercise tolerance, quality of life, and severity of depressive symptoms in GUCH patients long-term after surgical correction of CHD. METHODS: We studied 57 CHD patients (30 females, 27 males, mean age 23 ± 3.4 years) at least 12 months after surgical correction of a ventricular septal defect (VSD) or ostium secundum atrial septal defect (ASD II). All patients were offered a CCR program, and 31 of them participated (rehabilitation group [Reh]) and 26 refused (non-rehabilitated group [NReh]). All patients underwent baseline cardiopulmonary exercise testing (CPET) using a cycloergometer and a ramp protocol with an initial load of 20 W followed by 10 W load increments per minute. Psychological evaluation included the Beck Depression Inventory (BDI) and the Euro QoL 5D questionnaire to evaluate quality of life. Patients were reassessed 30 days after the initial evaluation using the same investigations. RESULTS: During CPET, all patients reached peak exercise intensity at the level of 15-17 in the Borg scale without complications. Resting HR was lower in the Reh group (74 ± 8 bpm) compared to the NReh group (81 ± 14 bpm). During CPET, patients in the Reh group reached significantly higher peak HR and percent maximum HR. Workload increased nonsignificantly (144 W vs. 124 W, p = 0.121), while duration of exercise and peak oxygen consumption was significantly longer in the Reh group compared to the NReh group (14 min vs. 11 min, p = 0.001; and 27.5 mL/kg/min vs. 23 mL/kg/min, p = 0.003, respectively). Patients in the NReh group showed non significantly higher severity of depressive symptoms compared to the Reh group, as evaluated using BDI at 30 days (mean score 4.8 vs. 2.2, respectively, p = 0.59). In contrast, subjectively and objectively rated quality of life was significantly higher in the Reh group compared to the NReh (score 89 vs. 74.4, p < 0.01; and 94 vs. 83, p < 0.01, respectively). CONCLUSIONS: Implementation of a CCR program improves physical capacity, exercise tolerance, and quality of life and reduces depressive symptoms in patients late after surgical correction of CHD. Introduction of such programs seems reasonable as a supplement to the holistic care for GUCH patients.

Cardiopulmonary exercise testing and SF-36 in patients with atrial septal defect type secundum.

PURPOSE: Although exercise capacity is impaired, atrial septal defect (ASD) patients report satisfactory exercise tolerance. This study aimed at (1) evaluating cardiopulmonary exercise testing (CPX) and (2) evaluating the impact of exercise capacity on perceived health status using a self-reported health questionnaire (SF-36) in patients with open and closed ASD. METHODS: Seventeen patients (mean age 37 ± 17, 9 males) with open ASD and 24 (mean age 37 ± 14, 6 male) with closed ASD were included. All underwent CPX and completed a SF-36 questionnaire. Age- and gender-matched controls were selected for comparison of CPX variables and SF-36 was compared with results from a general population. RESULTS: Patients with open ASD had lower peak oxygen uptake (VO(2)) (27.9 ± 9.7 vs 38.5 ± 9.5 mL · kg(-1) · min(-1). P = .009) and higher VE/VCO(2) slope (31.0 ± 7.7 vs 24.1 ± 4.8; P = .004) than controls. Patients with closed ASD had lower peak VO(2) (26.2 ± 8.4 vs 34.8 ± 9.7 mL · kg(-1) · min(-1). P = .014) and peak heart rate (163 ± 25 vs 178 ± 16 bpm; P = .035) than controls. Perceived health-status was lower in patients with open ASD than the general population. Peak VO(2) correlated significantly with physical functioning, emotional functioning, and bodily pain in open ASD and with physical functioning, bodily pain, role limitation, vitality, and mental health in closed ASD patients. CONCLUSION: Patients with open and closed ASD had decreased peak VO(2). Patients with open ASD had lower ventilatory efficiency. Closed ASD patients had chronotropic incompetence because of ß-blockers. SF-36 was reduced in patients with open ASD but not closed ASD. Reduced exercise capacity affected several domains of perceived health-status in ASD patients.

Cardiopulmonary exercise parameters in children with atrial septal defect and increased pulmonary blood flow: short-term effects of defect closure.

UNLABELLED: Markedly increased pulmonary blood flow because of a relevant atrial septal defect (ASD) leads to impaired cardiopulmonary function during maximum exercise in adults. No comparative preoperative and postoperative data are available on the short-term effects of shunt closure on cardiorespiratory function at peak exercise in children. Pulmonary function testing at rest and cardiopulmonary exercise testing together with haemodynamic assessment was done prospectively in children with an ASD preoperatively and again after full recovery at 3-4 mo postoperatively and compared with a matched normal population. Sixteen children, aged 6.8-16.1 y, with a defect of 8-23 mm (median 15 mm) and a pulmonary/systemic flow ratio of 1.5-3.5 (median 2.2) were tested and compared with 15 healthy children. Preoperatively, baseline pulmonary function parameters and exercise capacity were no different from normals. At peak exercise, patients with a shunt had increased pulmonary resistance, especially of the distal airways (p = 0.04), with a significantly larger proportion of children having a paradoxical increase in total airway resistance during exercise (p < 0.05). Maximum serum lactate at peak exercise was elevated (p < 0.05) in patients. In patients, maximum oxygen uptake was impaired (p = 0.03) and remained so at repeat evaluation postoperatively. The same observation was made for chronotropic response to exercise. CONCLUSION: Cardiopulmonary exercise parameters in patients with ASD differed only slightly from those in normal children. The most important deviations were a lower maximum oxygen uptake and an increase in airway resistance at maximum exercise.

[Conventional heart surgery with the fast-track-method: experiences from a pilot study]. / Konventionelle Herzchirurgie im Fast-track-Verfahren: Erfahrungen aus einer Pilotstudie.

Early release after cardiac surgery can be promoted by implementation of a standard protocol for accelerated perioperative and early postoperative care, with optimal education and support of the patient playing a key role. We report on our preliminary experience with 100 selected patients who underwent a "fast track" protocol following coronary artery bypass (n = 61), valve replacement or reconstruction (n = 34) or closure of an atrial septal defect (n = 5) between 1996 and 1998. Surgery was performed through a midline sternotomy using normothermic or mild hypothermic cardiopulmonary bypass. Patients undergoing cardiac surgery with less invasive techniques were excluded from this study. The following criteria had to be fulfilled for early hospital discharge: sinus rhythm, temperature below 37.5 degrees C, stable haematocrit around 0.30, uncomplicated wound healing and complete mobilisation including stair exercises. Mean duration of the operation was 137 +/- 24 minutes and mean intubation time was 4.5 +/- 3 hours. Mean duration of hospitalisation from the day of the operation was 4.9 +/- 2.1 days. There was no early or late mortality in this group of patients and only 2 patients had to be re-admitted on postoperative day 10 and 14 because of atrial fibrillation in one and a wound healing problem in the other. Accelerated recovery and early hospital discharge is highly attractive in selected patients; in helps to promote early cardiac rehabilitation and the costs of the procedure can be substantially reduced. According to our experience and the most recent literature, this approach does not expose patients to higher mortality or morbidity. In addition, fast-tracked patients have shown a higher level of satisfaction. Under optimal cooperation between surgery, anaesthesiology and intensive care unit, the fast-track protocol can be applied in approximately 30% of overall adult cardiac surgery patients.

The Holt-Oram syndrome: a case presentation and review of management parameters.

Holt-Oram syndrome (HOS) is a dominant inherited disorder which causes skeletal defects of the arms and heart disease. This report describes a 2-year-old child with HOS whose skeletal anomalies affected his functional capabilities and developmental sequence. A comprehensive evaluation of the child, which included radiographic, cardiac, gross and fine motor assessment, electrodiagnosis and consideration of surgery, resulted in a carefully formulated treatment plan. Review of the literature emphasizes the importance of selectively balancing conservative management with surgery for a satisfactory cosmetic result and optimal function.

[Late results and rehabilitation of patients with secondary interatrial septal defects]. / Otdalennye rezul'taty i reabilitatsiia bol'nykh s vtorichnymi defektami mezhpredserdnoi peregorodki.

The remote results were studied in 194 patients operated on for secondary atrial septal defects at ages of 18 months to 41 years. The follow-up period ranged from 5 months to 11 years. The ECG was normal in 74.7% of patients, its improvement was noted in 8.7% of patients. Disorders of rhythm were encountered in 10.3% of patients, their incidence being 2 1/2 times higher among adults than among children. The systolic murmur disappeared in 29.8% and diminished in 50.8% of patients. The diastolic murmur was corrected in 92.2% of cases. Splitting of the second sound disappeared in 66% and diminished in 6% of patients. The size of the heart on a radiogram became normal in 32.2% and its enlargement became less in 41% of the patients examined. The heart grows smaller within 2--3 years and the pulmonary pattern becomes normal in 1--2 years. The process is more rapid in children. The postoperative result was good in 90% of patients.