Unrepaired Ventricular Septal Defects Research Articles

Long-Term Outcomes of Adults With Isolated Unrepaired Ventricular Septal Defects in the Wellington Region

Long-Term Outcomes of Adults With Isolated Unrepaired Ventricular Septal Defects in the Wellington Region

Cardiopulmonary functional capacity in Taiwanese children with ventricular septal defects.

Children with ventricular septal defects (VSDs) are considered to have no difference in cardiopulmonary functional capacity with healthy children of the same age; however, studies have shown contradictory findings. The aim of this study was to assess whether Taiwanese children with VSDs exhibited cardiopulmonary deficits. This is a retrospective cohort study with the data collected from January 2010 to December 2021. All patients and controls (age-, sex-, and body mass index -matched) underwent cardiopulmonary exercise testing (CPET) and pulmonary function test. In total, 157 VSD patients (80 patients with surgically closed VSDs, 77 patients with unrepaired VSDs) and 157 healthy controls were recruited. Pulmonary function test showed significant among-group differences in maximal voluntary ventilation (MVV) (p=0.015). The surgically closed group had lower MVV compared to the control group. Regarding CPET, we found VSD patients had lower peak oxygen uptake than the controls (surgically closed group: 30.84±6.27ml/kg/min; unrepaired group: 32.00±5.95ml/kg/min; control group: 36.76±6.50ml/kg/min, p<0.001). There was also significant among-group differences in aerobic capacity (surgically closed group: 21.20±4.39ml/kg/min; unrepaired group: 21.68±4.47ml/kg/min; control group: 26.25±4.33ml/kg/min, p<0.001). In addition, the surgically closed group had lower heart rate average at anaerobic threshold than the control group (surgically closed group: 138.11±16.42 bpm; control group: 145.78±15.53 bpm, p=0.002). Taiwanese children with VSD, whether surgically closed or not, have poorer cardiopulmonary performance than age-matched healthy children, and the results of the surgically closed group were even worse.

Cardiac Arrhythmias and Impaired Heart Rate Variability in Older Patients With Ventricular Septal Defects.

BackgroundCongenital ventricular septal defects (VSDs) are considered to have benign long‐term outcome when treated correctly in childhood. However, abnormal parameters are described in younger adults, including impaired heart rate variability (HRV). It is not known whether such abnormalities will deteriorate with age. Therefore, HRV and cardiac events, such as premature ventricular contraction, were evaluated in patients aged >40 years with congenital VSDs and compared with healthy peers.Methods and ResultsA total of 30 surgically closed VSDs (51±8 years, repair at median age 6.3 years with total range 1.4–54 years) with 30 healthy controls (52±9 years) and 30 small, unrepaired VSDs (55±12 years) with 30 controls (55±10 years) were all equipped with a Holter monitor for 24 hours. Compared with healthy peers, surgically closed patients had lower SD of the normal‐to‐normal (NN) interbeat interval (129±37 versus 168±38 ms; P<0.01), SD of the average NN intervals for each 5‐minute segment of a 24‐hour HRV recording (116±35 versus 149±35 ms; P<0.01) and 24‐hour triangular index (31±9 versus 44±11; P<0.01). SD of the NN intervals, SD of the average NN intervals for each 5‐minute segment of a 24‐hour HRV recording, and triangular index were comparable between unrepaired VSDs and healthy peers. SD of the NN intervals was <100 ms in 22% of surgically closed and 10% of unrepaired VSDs, whereas controls were within normal ranges. A high number of premature ventricular contractions (>200 events) was registered in 57% of surgical patients compared with 3% of controls (P<0.01), and 53% of unrepaired VSDs compared with 10% in controls (P<0.01).ConclusionsAdults aged >40 with congenital VSDs demonstrate impaired HRV, mainly among surgically closed VSDs. More than half demonstrated a high number of premature ventricular contractions. These novel findings could indicate long‐term cardiovascular disturbances. This necessitates continuous follow‐up of VSDs throughout adulthood.

Infective endocarditis: prevention and antibiotic prophylaxis.

The Swiss societies of Infectious Diseases, Pediatric Cardiology and Cardiology and the Pediatric Infectious Disease Group of Switzerland present the current update on infective endocarditis prophylaxis in a joint initiative. The major focus of the revised recommendations is a comprehensive prevention campaign for all patients at risk for infective endocarditis. Antibiotic prophylaxis is recommended only for individuals at high risk. Within this high-risk group there is a ranking order, and the conditions are presented accordingly. Antibiotic prophylaxis is no longer recommended for patients with unrepaired ventricular septal defects and patent ductus arteriosus. Recommendations for antibiotic prophylaxis for the prevention of infective endocarditis are categorized in dental and non-dental interventions.

Comparison of Outcomes in Adults With Ventricular Septal Defect Closed Earlier in Life Versus Those in Whom the Defect Was Never Closed

Ventricular septal defects (VSDs) have recently demonstrated poorer functional outcome with disrupted ventricular contractility during exercise in young patients. It is not known whether this will change with age. Therefore, echocardiography was performed in older patients with congenital VSDs, either surgically closed or unrepaired and all without Eisenmenger physiology, to compare functional outcomes with healthy peers. Transthoracic echocardiography was performed at rest and during supine bicycle exercise, with tissue velocity Doppler for assessment of primary end points: isovolumetric acceleration and systolic velocities. In total, 30 surgically closed VSDs (51 ± 8 years) with 30 healthy controls (52 ± 9 years) and 30 unrepaired VSDs (55 ± 12 years) with 30 matched controls (55 ± 10 years) were included. Surgical patients displayed lower right ventricular (RV) systolic and early diastolic-filling velocities compared with controls, p <0.01, lower tricuspid annular plane systolic excursion (17.8 ± 5 vs 22.7 ± 3mm, p <0.01) and lower fractional area change (37.8 ± 6 vs 46.4% ± 7%, p <0.01). Unrepaired VSDs also had lower RV fractional area change than matched controls (39.9 ± 7 vs 48.4% ± 7%, p <0.01). Both patient groups had more tricuspid regurgitation and larger RV outflow tract dimensions than controls, p <0.01. During exercise, isovolumetric acceleration and systolic velocities were lower in both patient groups compared with controls, with the difference increasing with workload level. In conclusion, adults in their mid-50s with surgically closed or unrepaired VSDs have abnormal RV function at rest, with even more pronounced differences during exercise. These results suggest that a VSD, whether repaired early or considered hemodynamically insignificant, is not a benign lesion and continuous follow-up of adults is warranted.

Disappearance of the shunt and lower cardiac index during exercise in small, unrepaired ventricular septal defects.

Clinical studies have revealed decreased exercise capacity in adults with small, unrepaired ventricular septal defects. Increasing shunt ratio and growing incompetence of the aortic and pulmonary valve with retrograde flow during exercise have been proposed as reasons for the previously found reduced exercise parameters. With MRI, haemodynamic shunt properties were measured during exercise in ventricular septal defects. Patients with small, unrepaired ventricular septal defects and healthy peers were examined with MRI during exercise. Quantitative flow scans measured blood flow through ascending aorta and pulmonary artery. Scans were analysed post hoc where cardiac index, retrograde flows, and shunt ratio were determined. In total, 32 patients (26 ± 6 years) and 28 controls (27 ± 5 years) were included. The shunt ratio was 1.2 ± 0.2 at rest and decreased to 1.0 ± 0.2 at peak exercise, p < 0.01. Aortic cardiac index was lower at peak exercise in patients (7.5 ± 2 L/minute/m2) compared with controls (9.0±2L l/minute/m2), p<0.01. Aortic and pulmonary retrograde flow was larger in patients during exercise, p < 0.01. Positive correlation was demonstrated between aortic cardiac index at peak exercise and previously established exercise capacity for all patients (r = 0.5, p < 0.01). Small, unrepaired ventricular septal defects revealed declining shunt ratio with increasing exercise and lower aortic cardiac index. Patients demonstrated larger retrograde flow both through the pulmonary artery and the aorta during exercise compared with controls. In conclusion, adults with unrepaired ventricular septal defects redistribute blood flow during exercise probably secondary to a more fixed pulmonary vascular resistance compared with age-matched peers.

Cardiopulmonary dysfunction in adults with a small, unrepaired ventricular septal defect: A long-term follow-up

BackgroundThere are increasing reports of cardiac and exercise dysfunction in adults with small, unrepaired ventricular septal defects (VSDs). The primary aim of this study was to evaluate pulmonary function in adults with unrepaired VSDs, and secondly to assess the effects of 900 μg salbutamol on lung function and exercise capacity. MethodsYoung adult patients with small, unrepaired VSDs and healthy age- and gender-matched controls were included in a double-blinded, randomised, cross-over study. Participants underwent static and dynamic spirometry, impulse oscillometry, multiple breath washout, diffusion capacity for carbon monoxide, and ergometer bicycle cardiopulmonary exercise test. ResultsWe included 30 patients with VSD (age 27 ± 6 years) and 30 controls (age 27 ± 6 years). Patients tended to have lower FEV1, 104 ± 11% of predicted, compared with healthy controls, 110 ± 14% (p = 0.069). Furthermore, the patient group had lower peak expiratory flow (PEF), 108 ± 20% predicted, compared with the control group, 118 ± 17% (p = 0.039), and showed tendencies towards lower forced vital capacity and increased airway resistance compared with controls. During exercise, the patients had lower oxygen uptake, 35 ± 8 ml/min/kg (vs 47 ± 7 ml/min/kg, p < 0.001), minute ventilation, 1.5 ± 0.5 l/min/kg (vs 2.1 ± 0.3 l/min/kg, p < 0.001) and breath rate, 48 ± 11 breaths/min (vs 55 ± 8 breaths/min, p = 0.008), than controls. ConclusionAt rest, young adults with unrepaired VSDs are no different in pulmonary function from controls. However, when the cardiorespiratory system is stressed, VSD patients demonstrate significantly impaired minute ventilation and peak oxygen uptake, which may be early signs of parenchymal dysfunction and restrictive airway disease. These abnormalities were unaffected by the inhalation of salbutamol.

Reduced biventricular contractility during exercise in adults with small, unrepaired ventricular septal defects: an echocardiographic study.

Small ventricular septal defects are often considered to be without long-term haemodynamic consequences and so the majority remains unrepaired. However, we recently showed reduced functional capacity and altered right ventricular morphology in adults with small, unrepaired ventricular septal defects. The underlying mechanisms behind these findings remain unclear, and so, biventricular contractility during exercise was evaluated. Adults with small, unrepaired ventricular septal defects and healthy controls were examined with echocardiography during supine bicycle exercise with increasing workload. Tissue velocity Doppler was used for evaluating isovolumetric acceleration and systolic velocities during exercise. In total, 34 patients with ventricular septal defects, a median shunt- ratio of 1.2 (26 ± 6 years), and 28 healthy peers (27 ± 5 years) were included. Right ventricular isovolumetric acceleration was lower in patients as compared with controls at rest (97 ± 40 vs 158 ± 43 cm/s2, P = 0.01) and at peak heart rate (222 ± 115 vs 410 ± 120 cm/s2, P < 0.01). Peak systolic velocities were similar at rest, but differed with exercise (13 ± 3 vs 16 ± 3 cm/s, P = 0.02). Left ventricular isovolumetric acceleration was lower in patients as compared with controls throughout the test (P < 0.01). Septal isovolumetric acceleration was similar at rest, but reduced during increasing exercise as compared with controls (220 ± 108 vs 303 ± 119 cm/s2, P = 0.03). Left ventricular isovolumetric acceleration was negatively correlated with the shunt- ratio, and right ventricular and septal peak systolic velocities were positively correlated with lower functional capacity. Altered biventricular contractility is present during exercise in adults with small, unrepaired ventricular septal defects. These results add to the growing number of studies showing that long-term outcome in unrepaired ventricular septal defects may not be benign.

Does functional capacity depend on the size of the shunt? A prospective, cohort study of adults with small, unrepaired ventricular septal defects.

Small ventricular septal defects (VSDs) are considered to have great prognoses and most remain unrepaired. However, we have recently demonstrated lower functional capacity in patients with small VSDs compared with healthy peers. Using magnetic resonance imaging scans, we determined whether the functional capacity was correlated to the size of the shunt. We included patients with unrepaired VSDs and healthy adults between 18 and 40 years of age in a prospective, cohort study. Functional capacity was previously determined using an incremental bicycle test, establishing peak oxygen uptake. Magnetic resonance imaging scans were performed using a 1.5-tesla Philips scanner. With electrocardiographically triggered flow measurements, we calculated mean cardiac output from the pulmonary trunk and ascending aorta as well as vessel diameters. In total, 29 patients with unrepaired VSDs (26.5 ± 6 years) and 25 controls (26.9 ± 5 years) completed both studies. Previously measured peak oxygen uptake was nearly 20% lower in patients compared with controls (P = 0.002). All patients had shunt ratios below 1.5, with mean QpQs of 1.2 ± 0.1. When correlating shunt size to functional capacity, a negative correlation was found between the shunt ratio and peak oxygen uptake (r = −0.44, P = 0.020). Compared with controls, patients had increased forward and retrograde flow in the pulmonary trunk but comparable flows in the ascending aorta. Pulmonary diameter was also increased in patients (30.3 ± 4 mm) compared with controls (28.2 ± 3 mm; P = 0.041), whereas aortic dimensions were comparable. Our results demonstrate that, although small, unrepaired VSDs revealed reduced functional capacity that can be negatively correlated to the size of the shunt.

Small, unrepaired ventricular septal defects reveal poor exercise capacity compared with healthy peers: A prospective, cohort study

BackgroundSmall ventricular septal defects (VSDs) are considered to be without hemodynamic influence and most remain unrepaired. However, studies recently described late cardiac adverse consequences that could potentially affect functional capacity. Yet, this has never been assessed in adulthood. Therefore, the aim was to determine peak exercise capacity in adults with small VSDs compared with healthy, matched controls. MethodsIn a prospective, cohort study we included patients with unrepaired VSDs and healthy controls, (age 18 to 40years). Functional capacity was determined through incremental bicycle tests and gas exchange was measured breath-by-breath with Jaeger MasterScreen CPX®. Primary endpoint was peak oxygen uptake, while secondary endpoints were anaerobic threshold and health-related quality-of-life. ResultsIn total, 34 VSD patients (age 26.5±6years) and 28 controls (age 26.9±5years) were included. There were no differences between groups in demographic characteristics or habitual exercise levels. At peak exercise, patients reached lower peak oxygen uptake, 36.2±9ml/kg/min, compared with controls, 43.8±6ml/kg/min (p=0.002) along with lower maximal workload; patients 3.2±1 watt/kg and controls 3.8±1 watt/kg (p=0.001). Aerobic capacity was also poorer in patients, 24.5±8ml/kg/min compared with controls, 31.2±7ml/kg/min (p=0.005). Lastly, patients had lower health-related quality-of-life in terms of physical (p=0.017) and social functioning (p=0.003) compared with controls. In the patient group physical functioning was directly correlated to the impaired peak oxygen uptake (r=0.473, p=0.005). ConclusionWe demonstrated reduced subjective and objective functional capacity in small, unrepaired VSDs compared with controls. Furthermore, a correlation was seen between the impaired peak exercise capacity and lower self-estimated physical health.

Management of infants with large, unrepaired ventricular septal defects and respiratory infection requiring mechanical ventilation

Objectives We sought to describe the hospital management and early outcome of critically ill infants presenting with large ventricular septal defects and pneumonia requiring mechanical ventilation at a referral center in a developing country. Infants with large ventricular septal defects who have pneumonia might present with respiratory failure requiring mechanical ventilation. In the developing world this presentation is relatively common, but few data exist describing patient management strategies. Methods Hospital data of consecutive infants admitted with large ventricular septal defects and pneumonia requiring mechanical ventilation were reviewed and analyzed. Results We identified 18 infants (mean age, 3.6 ± 3.0 months). On admission, all the infants were significantly malnourished, and echocardiography showed bidirectional shunting (predominantly right-to-left shunting) in 6 infants. Thirteen (72%) patients improved with intensive medical management that included mechanical ventilation for 1 to 16 days (median, 6.5 days); unequivocal left-to-right shunting was subsequently documented by means of echocardiography in all 13 patients. Twelve patients underwent surgical repair, and 11 (91.6%) were discharged after median mechanical ventilation of 100 hours (range, 42-240 hours) and intensive care unit stay of 8 days (range, 4-15 days). Five of 6 unoperated patients died, 4 of them within a few hours of admission. One child with multiple ventricular septal defects was discharged and subsequently underwent pulmonary artery banding. Conclusion Corrective cardiac surgery for selected critically ill infants with large ventricular septal defects, severe malnutrition, and pneumonia requiring mechanical ventilation is feasible and should be considered a viable management strategy.

Total but not resting energy expenditure is increased in infants with ventricular septal defects.

The purpose of this study was to determine the effect of left-to-right shunting on the resting energy expenditure (REE), total energy expenditure (TEE), and energy intake in a group of 3- to 5-month-old infants with moderate to large unrepaired ventricular septal defects (VSDs) compared with age-matched, healthy infants. Eight infants with VSDs and 10 healthy controls between 3 to 5 months of age participated in the study. Indirect calorimetry was used to measure REE and the doubly-labeled water method was used to measure TEE and energy intake. An echocardiogram and anthropometric measurements were performed on all study participants. Daily urine samples were collected at home for 7 days. Samples were analyzed by isotope ratio mass spectrometry. Data were compared using analysis of variance. No significant differences were found in REE (VSD, 42.2 +/- 8.7 kcal/kg/d; control, 43.9 +/- 14.1 kcal/kg/d) or energy intake (VSD, 90.8 +/- 19.9 kcal/kg/d; control, 87.1 +/- 11.7 kcal/kg/d) between the groups. The percent total body water was significantly higher in the VSD infants and the percent fat mass was significantly lower. TEE was 40% higher in the VSD group (VSD, 87.6 +/- 10.8 kcal/kg/d; control, 61.9 +/- 10.3 kcal/kg/d). The difference between TEE and REE, reflecting the energy of activity, was 2.5 times greater in the VSD group. REE and energy intake are virtually identical between the two groups. Despite this, infants with VSDs have substantially higher TEE than age-matched healthy infants. The large difference between TEE and REE in VSD infants suggests a substantially elevated energy cost of physical activity in these infants. These results demonstrate that, although infants with VSDs may match the energy intake of healthy infants, they are unable to meet their increased energy demands, resulting in growth retardation.