Per G. Bjørnstad

Mental health and psychosocial functioning in adolescents with congenital heart disease. A comparison between adolescents born with severe heart defect and atrial septal defect

Twenty‐six adolescents, aged 13–18 years, with severe congenital heart disease were matched for sex, age and living area with 26 adolescents with repaired atrial septal defect and regarded as physically fit. These two groups were compared according to somatic condition, psychopathology, psychosocial functioning and chronic family difficulties. A higher rate of psychiatric problems in the complex group, an association between psychosocial functioning and physical capacity, as well as an association between psychosocial functioning and chronic family difficulties were observed. These findings suggest that physical capacity is of crucial importance for mental health and functioning of adolescents with congenital heart disease. The association with chronic family difficulties also suggests that a comprehensive biopsychosocial approach is necessary in the treatment and rehabilitation of these patients.

Cardiac findings in generalized lipodystrophy

The cardiac findings in eight patients, two of whom were female, with total lipodystrophy (Berardinelli‐Seips disease) are reported. One of them had the acquired form of the disease. Four patients died at a mean age of 32 years. As far as we know, at least three of them most likely died for cardiac reasons, one shortly after recovering from an attempted suicide. All eight patients had hypertrophic hearts, mostly with deranged diastolic, but also systolic, function. One had pulmonary hypertension. We conclude that generalized lipodystrophy is a serious disease with cardiac affection leading to cardiac dysfunction and early death. There is no specific cardiac treatment, and the treatment should be according to the general guidelines for patients with hypertrophic, dysfunctioning hearts.

Reduced pulmonary function in children with the Fontan circulation affects their exercise capacity

Most children with functionally univentricular hearts nowadays are treated surgically by creating a total cavopulmonary connection. In the resulting Fontan circulation, the venous return and the pulmonary arterial bed are coupled in series, bypassing the heart. This gives the potential for interaction between the abnormal circulation and function of the lungs. In this study, we investigated the pattern of impairment of pulmonary function, and its relation to decreased exercise capacity. We performed spirometry in 33 (85 percent) of 39 eligible Norwegian children, aged from 8 to 16, with a total cavopulmonary connection, along with whole body plethysmography, the carbon monoxide single breath test, and a peak treadmill exercise test. The single breath test showed a mean corrected diffusing capacity of 66.5 percent of predicted, giving a z score of minus 2.88. The mean residual volume measured by whole body plethysmography was 146.8 percent, equivalent to a z score of 2.46, whereas the mean residual volume measured by the single breath test was 102.4 percent of predicted, this being the same as a z score of 0.43. The mean peak treadmill exercise test was 70.0 percent of predicted, equivalent with a z score of minus 3.07. Mean forced vital capacity was 85.7 percent of predicted, the equivalent z score being minus 0.92. Lung function correlated with the peak treadmill exercise test. We have shown, therefore, that children with the Fontan circulation have reduced diffusing capacity, possibly caused by the abnormal circulation through the lungs. The difference between residual volume measured by plethysmography and the single breath test implies trapping of air. The correlation of parameters for lung function with peak consumption of oxygen during exercise indicates that the abnormalities of pulmonary function may affect physical capacity.

The impact of severe congenital heart disease on physical and psychosocial functioning in adolescents

A study was made of 26 adolescents, aged from 13 to 18 years, with different types of severe congenital heart disease, assessing their somatic condition and its impact on mental health and psychosocial functioning. Physical capacity was reduced, more pronounced in girls than in boys, along with an increased rate of psychiatric problems. Associations were found between physical capacity and psychosocial functioning, and between psychosocial functioning and chronic family difficulties. On the other hand, half the patients studied achieved fair function, both physically and with regard to their mental health. The degree of reduced physical capacity is an important etiological factor for impaired mental health in patients with congenital heart disease. This knowledge should be taken into account when dealing with this group of patients. A good social network seems to be a protective factor. It will probably be rewarding to give patients and their families not only an optimal medical follow-up, but also psychological, practical and financial support to improve or create such a network.

The Amplatzer ® Membranous VSD Occluder and the vulnerability of the atrioventricular conduction system

Transcatheter closure of ventricular septal defects with the Amplatzer Membranous VSD Occluder has yielded promising initial results, but disturbances of conduction, including complete heart block, have been reported. We report our experience with the Amplatzer occluder in 35 patients with a median age 4.5 years, the defects being sized angiographically at 4.4 plus or minus 1.1 millimetres, with a range from 3 to 8 millimetres, and the size of the occluder varying from 4 to 12 millimetres. Over a median follow-up of 2.5 years, the rate of complete closure was 87% and 91%, at 1 and 2 years respectively, while 2 patients required surgical closure of the defect subsequent to the insertion of the device. Persistent regurgitation across the tricuspid valve related to the occluder was observed in 3 patients, and in 6 patients across the aortic valve. Abnormalities of conduction related to the procedure were noted in 7 patients, one-fifth of the cohort. The disturbances were transient in 1 patient, but permanent in 6, in one of the latter progressing after 6 months from left bundle branch block to intermittent Mobitz II second-degree atrioventricular block in association with expansion of the occluder. We conclude that transcatheter closure of perimembranous ventricular septal defects with the Amplatzer occluder is effective with limited complications, but the incidence of immediate and progressive disturbances of conduction related to the proximity of conduction tissues to the rims of the occluder stress the importance of larger and longer studies to assess the safety of this procedure.

Truncus arteriosus with anomalous pulmonary venous connection

The anatomic, diagnostic, and management findings of 6 patients with truncus arteriosus and anomalous pulmonary venous connections are described. Additional risk factors indicative of poor prognosis were found in 3 of 4 patients with truncus arteriosus and totally anomalous pulmonary venous connection and in 1 patient with partially anomalous pulmonary venous connection.

The impact of shunt size on lung function in infants with univentricular heart physiology.

Objectives: To assess the relationship that shunt size, blood gases, and radiologic findings has on respiratory function in infants with univentricular heart physiology. Setting: Cardiac catheter laboratory at Rikshospitalet University Hospital, Norway. Patients: Fifteen infants with univentricular heart physiology admitted for cardiac catheterization. Measurements: Lung function was measured by a fixed-orifice differential pressure flow sensor and mainstream volumetric capnography in 15 infants with univentricular heart arrangements during routine invasive assessment before the bidirectional cavopulmonary connection. Blood gases were measured from the indwelling catheters. Chest radiographs were assessed for heart size and pulmonary vasculature. Shunt size was assessed angiographically. Main Results: Respiratory compliance was reduced in patients with a large surgical systemic-pulmonary arterial shunt (r = −0.67, r2 = 0.45, p = 0.03). Respiratory resistance was higher with increased heart size (r = 0.72, r2 = 0.52, p = 0.004). There was no association between arterial and end-tidal CO2 values. The arterial to end-tidal CO2 difference had an inverse relationship with the pulmonary to systemic shunt ratio (r = −0.38, r2 = 0.14, p = 0.015). Conclusion: A large surgical shunt size is related to stiffer lungs and a large heart is associated with a higher respiratory resistance. During mechanical ventilation of patients with univentricular heart physiology the end-tidal CO2 may be an unreliable substitute for arterial CO2 before the bidirectional cavopulmonary connection. We found a relationship between a decreased pulmonary to systemic shunt ratio and an increased arterial to end-tidal CO2 difference. This may indicate that a reason for the unreliability of end-tidal CO2 is an impaired gas exchange partially due to pulmonary hypoperfusion.

Transcatheter closure of atrial septal defects in the oval fossa: is the method applicable in small children?

We report our experience from 1996 through 1999, representing our initial experience with use of the Amplatzer device to close atrial septal defects. Of 46 patients taken to the catheter laboratory with the intention to close the defect, the device was permanently implanted in 40 (87%). They were aged between 1.4 and 71.8 years, with weights ranging from 7.8 to 90 kg. Both age and weight distributed into two peaks, demonstrating two different populations. The size of the devices, taking the biggest device if two were inserted, was between 9 and 30 mm. We underwent a short learning curve, but the time required for fluoroscopy, or the number of difficulties experienced, showed no connection with the size or age of the patient, nor the size of the defect itself. A suspicion that young age and small size would increase the risk and difficulties, and result in more interrupted procedures, could not be substantiated. In children no interruption was procedural. Our early experience, therefore, demonstrates that an experienced interventional team can use the Amplatzer occluder successfully to close atrial septal defects in patients of all ages and sizes, at least from 7.8 kg and up.

Catheter-based closure of atrial septal defects in the oval fossa with the Amplatzer® device in patients in their first or second year of life†

Objective: To assess feasibility, safety, and efficacy of the use of Amplatzer® occluders in closure of atrial septal defects (ASD) in children in the first 2 years of life. Background: Although scattered reports on such closure have been published, no agreement consists on the policy. Methods: In 654 patients of all ages, closure was achieved in 632 (96.6%). Data were analyzed retrospectively in all 71 children where an attempt had been made to close the ASD before their second birthday. Results: Median age and body weight were 17.2 months (range 3.9–23.8) and 10.0 kg (range 3.8–14.5), respectively. Median fluoroscopy time was 13.6 min and median device size 15 mm. Successful closure was achieved in 68 children (95.8%). Three times the procedure was aborted: in one, the device repeatedly straddled the septum; in the other two, a small left atrium restricted the movement of the left‐sided disc. One device embolized and was reimplanted after retrieval. One infant with multiple disorders died 6 days after closure from acute sepsis probably unrelated to the procedure. No other complications occurred. Only trivial shunts closing with time were registered during follow‐up. Symptomatic patients profited markedly from closure. Conclusion: The results and complications of ASD closure with the Amplatzer® device in patients in their first 2 years of life compare favorably with procedures in older patients, provided that the size of the septum and the dimensions in the left atrium are taken into consideration when selecting the size of the device.

Balloon debanding of the pulmonary artery.

Clinical balloon debanding of the pulmonary artery is reported in two patients, 6 months and 4 years of age. The band was made of Dacron, and was closed with one single polypropylene suture. This suture was subsequently ruptured with a balloon inflated within the pulmonary artery at the site of the band.Balloon debanding relieved the gradient almost completely in the two patients. No problems occurred during the procedure. In the future this may prove to be an efficient and reliable way to remove the effect of a pulmonary artery banding.