Asian Cardiovasc Thorac Ann 2000;8:38-40
© 2000 Asia Publishing EXchange Pte Ltd
Closure of Isolated Secundum Atrial Septal Defects in Infancy
Raju S Iyer, MCh,
Andreas Hoschtitzky, MD,
Jefferey Jacobs, MD,
Martin Elliott, MD,
Marc de Leval, MD,
Jaroslav Stark, MD
Cardiothoracic Unit
The Hospital for Sick Children
London, UK
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For reprint information contact: Raju S Iyer, MCh Tel: 91 422 21 1000 Fax: 91 422 21 3509 email: gknmh{at}vsnl.com Department of Cardiothoracic Surgery, G Kuppuswamy Naidu Memorial Hospital, P.O. Box 6327, Pappanaickenpalayam Avinashi Road, Coimbatore, Tamil Nadu 641037, India.
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Abstract
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Between 1965 and 1995, 552 patients underwent closure of isolated secundum atrial septal defect, of whom 24 (4.3%) were infants with a mean age of 238.5 ± 13.8 days (range, 90 to 348 days). Mean weight was 6 ± 0.3 kg (range, 3.5 to 9 kg). Twenty-two had noted failure to thrive and 13 had chest infections. Direct closure was carried out in 20 and 4 underwent patch closure. There were 3 (12.5%) early deaths (intraoperatively, 5 hours, and 2 days postoperatively). Three patients had pulmonary hypertension with pulmonary arterial to systemic arterial pressure ratios of 0.74 to 0.83 preoperatively. Of 21 survivors, 13 were extubated within 24 hours and 8 within 72 hours of surgery. Mean hospital stay was 16.2 ± 8.2 days. There were 2 late deaths; one from pneumonia at 177 days postoperatively and another patient with a severe neuromuscular disorder who could not be extubated, died 328 days postoperatively. We concluded that some infants require early surgery and an atrial septal defect can be closed safely in infancy but the risk increases in patients with pulmonary vascular disease. The etiology of pulmonary vascular disease in such patients remains unclear.
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Introduction
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One of the most common congenital cardiac anomalies is isolated atrial septal defect (ASD). Untreated, it usually follows a benign course throughout the first 2 decades of life. Most cases of isolated ASD are surgically treated between 3 and 8 years of age. Usually, patients do not present clinically in the first year of life.1–6 Closure of an isolated ASD in infancy is rare and only a few reports have described small groups of patients needing surgical closure in the first year of life.1,2,7–9 The outcome in these reports varies considerably. To determine whether this patient subgroup has characteristics that differentiate it from the general population of patients with isolated secundum ASD, we reviewed all cases of isolated secundum ASD in which surgical closure was carried out in the first year of life at The Hospital for Sick Children, London, UK.
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Patients and Methods
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Between 1965 and 1995, 552 patients underwent closure of isolated secundum ASD, of whom 24 (4.3%) were infants. Thirteen were female, 10 were male, and 1 was of ambiguous gender. The records of the initial and follow-up data were reviewed retrospectively. Patients with ostium primum ASD, mitral insufficiency, anomalous pulmonary venous drainage, or other associated intra-cardiac lesions were excluded from this study. Data were collected pertaining to clinical symptoms, 12-lead electrocardiograms, chest radiography, echocardiography, cardiac catheterization (if any), and the surgical reports. Follow-up data were provided by the patients' general physicians. All patients were followed up bimonthly for the first 6 postoperative months and then annually.
The majority of these infants (22/24) presented with failure to thrive (Table 1
). One patient was asymptomatic but she underwent surgery at the same time as a sibling with ASD, by parental request. Three patients had been born prematurely and 7 presented in the first week of life. The mean age at onset of symptoms was 9 weeks (range, 0 to 28 weeks). The mean age at operation was 238.5 ± 13.8 days (range, 90 to 348 days). The mean weight at operation was 6 kg (range, 3.5 to 9 kg). The weights and heights are compared with the 50th percentile of normal in Table 2. Physical examination revealed tachypnea in 11 patients, cyanosis in 2, and congestive cardiac failure in 17. An ejection systolic murmur was found in 16, a continuous murmur in 2, a mid-systolic murmur in 2, and 4 patients had no murmur on cardiac auscultation. Chest radiography demonstrated cardiomegaly in 16 cases. Electrocardio-graphy showed signs of right ventricular hypertrophy in 17 and varied rhythm disturbances in 6 patients (3 with incomplete right bundle branch block and one each with first-degree heart block, supraventricular tachycardia, and unspecified tachycardia). Echocardiography data were available in 18 patients, most of whom showed right ventricular volume overload and a secundum ASD. The ASD was noted as large (more than 8 mm) in 16 patients and moderate (3 to 8 mm) in 8. Cardiac catheterization was performed in 9 cases of suspected pulmonary hypertension; the mean pulmonary vascular resistance was 4 Wood units (range, 1 to 6 Wood units), the mean pulmonary to systemic flow ratio was 2.6 with a range of 2 to 3.5. Eight patients had associated patent ductus arteriosis, 2 had a small ventricular septal defect, and 2 had a left superior vena cava. Twenty infants underwent primary closure of the defect and 4 had patch closure. In patients with associated patent ductus arteriosis, the ductus was ligated.
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Results
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Thirteen patients (54%) were extubated within 24 hours and 8 were extubated between 24 and 72 hours after surgery; 2 had delayed sternal closure and 1 was re-intubated. Reexploration was required in 4 cases because of residual ASD, cardiac arrest, chylous leak, or sternotomy wound infection. There were 3 (12.5%) early deaths (intraoperatively, 5 hours; and 2 days post-operatively) and 2 late deaths (overall mortality, 20.8%). Of the 3 patients who died early, 1 required preoperative mechanical ventilation and died from massive pneumonia unresponsive to antibiotics (pulmonary vascular resistance was not measured in this patient). The second patient had a pulmonary hemorrhage, bradycardia, and hypoxia and died due to pulmonary hypertensive crisis, and the third also succumbed to pulmonary hypertensive crisis; these patients had pulmonary arterial to systemic arterial pressure ratios of 0.74 to 0.83. Postmortem examination in the 2nd and 3rd patients revealed grade III and IV changes of pulmonary hypertension. One of the late deaths was from pneumonia at 177 days postoperatively and the other patient who had a severe neuromuscular disorder and could not be extubated, died 328 days postoperatively. Mean hospital stay of the other patients was 16.2 ± 8.2 days. Review of the follow-up of the surviving patients showed that 11 were not taking any medication, digoxin and diuretics were continued in 6, and 2 were lost to follow-up. The improvement in weight and height postoperatively is illustrated in Table 3. The mean duration of follow-up was 5 years.
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Discussion
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Some infants with isolated secundum ASD fail to thrive and require early surgery.1,3,5,6,10,11 Most of those who present early are symptomatic and have a wide variety of murmurs. Interestingly, a large number of these patients had dysmorphic features. As this group consisted of a very wide spectrum of chromosomal and congenital anomalies, no definitive conclusion could be reached. The majority had failed to thrive and the predominant symptom was recurrent respiratory tract infection. Previous studies have indicated that this form of secundum ASD has a good prognosis, although these have mainly involved small numbers of patients mostly over 1-year-old.8 The patients in this study were all under one year of age and probably more severely ill with congestive cardiac failure, which may account for the slightly higher mortality rate. As stated by Bull and colleagues,1 children with large shunts run the risk of developing pulmonary vascular disease early. Eventually, pulmonary vascular disease leads to pulmonary hypertensive crisis and increases the risk of surgery.4
Large ASDs have a low incidence of spontaneous closure and hence early surgical closure is adopted in these cases.12 Failure to control the symptoms with medical treatment ultimately leads to the need for surgical closure. In cases of preexisting pulmonary hypertension, a valved atrial patch is a possible method of surgical closure.13 The percentiles for height and weight before and after surgery (Tables 2 and 3
) demonstrated a marked improvement postoperatively, with both percentiles reaching near normal values.
The 2 patients who died from pulmonary hypertensive crisis had high pulmonary vascular resistance (5.5 and 6 Wood units) on cardiac catheterization. Both had persistent hypoxia in spite of ventilation with a high fraction of inspired oxygen and other measures to control the crisis. The 6 patients who were still being treated with digoxin and diuretics were on tapering doses of these drugs for 2 years because of congestive cardiac failure post-operatively. Our mortality appears high but most of the deaths were concentrated in the early part of the series.
The protocol for management of pulmonary hypertensive crisis included: monitoring pulmonary artery and left atrial pressures; stabilizing blood gases and pH; avoiding pain, tracheal suctioning, lung infections, sepsis, and early weaning from the ventilator; taking note of early signs such as high pulmonary arterial and central venous pressures, low systemic and left atrial pressures, low O2 saturation, tachycardia, and low cardiac output. Prevention of crisis involved sedation with fentanyl (4 to 8 µg•kg–1•h–1), paralysis with vecuronium (3 µg•kg–1•min–1), hyperventilation and high FiO2 (fraction of inspired oxygen) to maintain PCO2 at 25 to 30 mm Hg and PO2 above 100 mm Hg, and infusion of phenoxybenzamine (0.5 mg•kg–1 intravenously, 8 hourly) usually started in the operating theatre. Treatment of crisis included hand ventilation with 100% O2, increasing sedation (fentanyl 8 µg•kg–1•h–1), infusion of prostacyclin at 5 to 20 ng•kg–1•min–1 via the pulmonary arterial line, and glyceryl trinitrate at 5 to 8 µg•kg–1•min–1. Inhaled nitric oxide was also part of the drug protocol, being attached to the ventilator circuit. After resolution of the crisis, support was reduced by gradually decreasing FiO2 while maintaining PO2 of 100 mm Hg and allowing PCO2 to rise slowly to 30 to 35 mm Hg. When the problem resolved, paralysis was discontinued, fentanyl was stopped, and the patient was then weaned from inotropic agents and vasodilators.
The conclusions drawn from this study were that some infants with isolated ASD fail to thrive and require early surgical closure. If left untreated, rapid development of pulmonary hypertension is likely and the risk of surgery increases in the presence of pulmonary hypertension. Associated chromosomal or congenital anomalies could have a direct effect on the prognosis of such patients. The etiology of the rapid progression of pulmonary vascular disease in such sick infants remains unclear.
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References
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Bull C, Deanfield J, de Leval M, Stark J, Taylor JFN, McCartney FJ. Correction of isolated secundum atrial septal defect in infancy. Arch Dis Child 1981;56:784–6.[Abstract/Free Full Text]
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Dimich I, Steinfeld L, Park SC. Symptomatic atrial septal defect in infants. Am Heart J 1973;85:601–4.[Medline]
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Hastreiter AR, Wennemark JR, Miller RA, Paul MH. Secundum atrial septal defect with congestive heart failure during infancy and early childhood. Am Heart J 1962;64:467–72.
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Haworth SG. Pulmonary vascular disease in secundum atrial septal defect in childhood. Am J Cardiol 1983; 51:265–72.[Medline]
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Hunt CE, Lucas RV. Symptomatic atrial septal defect in infancy. Circulation 1973;47:1042–8.[Abstract/Free Full Text]
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Weinberg M, Miller RA, Hastreiter AR, Raffensperger JG, Fell EH, Bucheleres HG. Congestive heart failure in children with atrial septal defect. J Thorac Cardiovasc Surg 1966;51:81–7.[Medline]
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Phillips SJ, Okies JE, Henken D, Sunderland CO, Starr A. Complex of secundum atrial septal defect and congestive heart failure in infants. J Thorac Cardiovasc Surg 1975;70:696–700.[Abstract]
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Phillips SJ. Secundum atrial septal defect in infancy. J Thorac Cardiovasc Surg 1976;72:816–7.[Medline]
-
Spangler JG, Feldt RH, Danielson GK. Secundum atrial septal defect encountered in infancy. J Thorac Cardiovasc Surg 1976;71:398–401.[Abstract]
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Ainger LE, Pate JW. Ostium secundum atrial septal defect and congestive heart failure in infancy. Am J Cardiol 1965;15:380–5.
-
Nakamura FF, Hauck AJ, Nadas AJ. Atrial septal defects in infants. Pediatrics 1964;34:101–6.[Abstract/Free Full Text]
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Radzik D, Davignon A, Van Doesburg N, Fournier A, Marchand T, Ducharme G. Predictive factors for spontaneous closure of atrial septal defects diagnosed in the first 3 months of life. J Am Coll Cardiol 1993;22: 851–3.[Abstract]
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Leyro-Diaz RM. Valved atrial septal patch. J Thorac Cardiovasc Surg 1996;111:285–6.[Free Full Text]
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Abstract
Introduction
