Septal Defect In Infants Research Articles

Rudhe syndrome: reversible right middle lobe emphysema in infants with left-to-right shunts—an historical review

In 1971, the Swedish radiologist Ulf Rudhe wrote a provocative paper on right middle lobe emphysema in infants with left-to-right shunts in which he suggested cardiac surgery rather than lung resection. At the time, this was counter to accepted medical practice. Earlier diagnosis and better medical management of ventricular septal defect in infants has proved Rudhe correct. However, two current cases of large left-to-right shunts in infants with emphysema of the right middle lobe prompt this historical review of what seemed a closed-episode in pediatric cardiac surgery.

Frequency of Severe Pulmonary Hypertension Complicating “Isolated” Atrial Septal Defect in Infancy

Frequency of Severe Pulmonary Hypertension Complicating “Isolated” Atrial Septal Defect in Infancy

Complete heart block associated with device closure of perimembranous ventricular septal defects

The development of the Amplatzer Membranous VSD Occluder (AGA Medical Corp, Plymouth, Minn) for closure of the perimembranous ventricular septal defect has ameliorated many of the technical difficulties of previous devices. Application of this new technology requires comparative evaluation with the current standard of surgical repair. We report our experience of complete heart block associated with device closure of a large perimembranous ventricular septal defect with unequivocal indications for intervention. We performed a retrospective review of 20 patients between January 2003 and August 2005 who underwent perimembranous ventricular septal defect device closure, 18 with hemodynamically large shunts meeting the surgical criteria for intervention. The median age was 1.6 years (range, 0.5-16.2 years), and the median weight was 9.7 kg (range, 6.2-43 kg). Acute complete shunt occlusion was achieved in all patients. There were no acute procedural complications. The median follow-up time was 23.1 months (range, 1-37.8 months). Four (22%) had complete heart block at 17 days, 4.2 months, 8.8 months, and 37.5 months after implantation, respectively. No risk factors were identified for development of complete heart block, including age, weight, trisomy 21, preceding conduction abnormalities, perimembranous ventricular septal defect size related to body surface area or device size, and progressive device flattening. Device closure of large perimembranous ventricular septal defects in infants and children with the Amplatzer Membranous VSD Occluder resulted in excellent closure rates but an unacceptably high rate of complete heart block.

Frequency of Severe Pulmonary Hypertension Complicating “Isolated” Atrial Septal Defect in Infancy

Atrial septal defects (ASDs) are typically asymptomatic in infancy and early childhood, and elective defect closure is usually performed at ages of 4 to 6 years. Severe pulmonary hypertension (PH) complicating an ASD is seen in adulthood and has only occasionally been reported in small children. A retrospective study was undertaken to evaluate the incidence of severe PH complicating an isolated ASD and requiring early surgical correction. During a 10-year period (1996 to 2006), 355 pediatric patients underwent treatment for isolated ASDs either surgically or by catheter intervention at 2 tertiary referral centers. Two hundred ninety-seven patients had secundum ASDs, and 58 had primum ASDs with mild to moderate mitral regurgitation. Eight infants were found with isolated ASDs (6 with secundum ASDs and 2 with primum ASDs) associated with significant PH, accounting for 2.2% of all patients with ASDs at the centers. These 8 infants had invasively measured pulmonary artery pressures of 50% to 100% of systemic pressure. They were operated in the first year of life and had complicated postoperative courses requiring specific treatment for PH for up to 16 weeks postoperatively. The ultimate outcomes in all 8 infants were good, with persistent normalization of pulmonary pressures during midterm follow-up of up to 60 months (median 28). All other patients with ASDs had normal pulmonary pressures, and the mean age at defect closure was significantly older, at 6.2 years for secundum ASDs and 3.2 years for primum ASDs. In conclusion, ASDs were rarely associated with significant PH in infancy but then required early surgery and were associated with excellent midterm outcomes in these patients.

Is Left Ventriculotomy Feasible for Muscular Ventricular Septal Defects in Infants?

Optimal management of muscular ventricular septal defects (MVSDs) remains controversial. Left ventriculotomy is the cornerstone of surgical repair but is frequently complicated by residual shunts, left ventricular dysfunction, apical aneurysm, or arrhythmias. In this study, we evaluated the long-term outcomes of surgical repairs in infants with isolated MVSDs. We retrospectively analyzed clinical data from 56 children with MVSDs (31 males, 25 females). Follow-up by questionnaire and Doppler echocardiography was performed at discharge and between 2 and 124 months after surgery. Patient age was 2 to 40 months (median, 21 months) and weight was 3.0 to 15.3 kg (median, 5.3 kg). Two patients died after surgery (hospital mortality, 3.57%). One patient with MVSDs died of low cardiac output caused by the long duration of cardiopulmonary bypass. Another patient with Swiss cheese MVSD received a single patch closure but died of low cardiac output immediately after cardiopulmonary bypass. Immediate complications such as a third-degree atrial-ventriclar block occurred in 2 patients, but they recovered before discharge and showed no residual shunt. No deaths occurred during follow-up, but a residual shunt was found in 1 patient. Delayed complete heart block requiring a pacemaker occurred in 1 patient. One patient showed paroxysmal supraventricular tachycardia that was treated with amiodarone. The left ventricular ejection fraction was 0.45-0.55 in 8 patients and 0.55-0.73 in 46 patients. No apical aneurysm was found. All the surviving patients returned to normal school life. Our results indicate that surgery is a suitable treatment option in infants and children with isolated MVSDs and that preoperative diagnosis is crucial to a successful outcome. Infants can tolerate a left ventriculotomy incision for MVSDs in the lower or apical ventricular septum.

Surgical treatment for ventricular septal defect in infants under 5 kg of body weight

To review the indication and timing of surgery,surgical techniques and perioperative management of ventricular septal defect (VSD) repair on infants under 5 kg of body weight. From January, 2000 to December, 2005, 134 children patients with VSD and associated anomalies, who were under 5 kg of body weight and aged (3.9+/-1.9) months, were diagnosed and treated in our department. One case died of arrhythmia and heart failure in the early stage of postoperation (mortality 0.7%). The most common postoperative complications were pneumonia (14), pulmonary hypertensive crisis (6), arrhythmia (4), low cardiac output (4), dropsy of thoracic cavity(1). Followed up for 6 m-6 y, the development of all 133 cases was well. Two children with residual shunt got a spontaneous closure confirmed by echocardiography one year postoperatively. The clinical results of operations on VSD infants with low body weight are satisfactory with attention to the indication and timing of surgery, proper CPB management, rational operations and perioperative management.

New transcatheter techniques for creation or enlargement of atrial septal defects in infants with complex congenital heart disease

To describe a series of 8 consecutive infants (5 with transposition of the great arteries [TGA] and 3 with hypoplastic left heart syndrome [HLHS]) who underwent nonconventional septostomy techniques. For some complex congenital heart defects, an unrestrictive atrial septal defect (ASD) is essential to achieve an adequate cardiac output and/or systemic saturation. In some scenarios, the use of conventional septostomy techniques may be technically difficult, hazardous, and/or ineffective. Use of transhepatic approach, cutting balloons, and radiofrequency perforation with stenting of the atrial septum. The size of the ASD and the oxygen saturation increased in all patients with no major complications. In those with TGA, the ASDs were considered to be of good size at the arterial switch operation. Two of the 3 patients with hybrid palliation for HLHS have developed some degree of obstruction within the interatrial stent over 2-3 months. At surgery, the stents were found to be secured within the septum with one showing significant fibrous ingrowth after uneventful removal. The other had some nonobstructive ingrowth. Creation or enlargement of ASDs in infants using new nonconventional transcatheter techniques is feasible, safe, and effective, at least in the short-to-mid-term follow-up. Infants with TGA seem to benefit the most because the procedure results in satisfactory clinical stability for subsequent early surgical intervention. In infants with HLHS palliated by a hybrid approach, stent implantation to the atrial septum seems to buy enough time to bring them to the phase II safely despite progressive in-stent obstruction.

Transcatheter Closure of Perimembranous Ventricular Septal Defects in Infants and Children Using the Amplatzer Perimembranous Ventricular Septal Defect Occluder

There are very few published reports of the transcatheter closure of perimembranous ventricular septal defects (PMVSDs) using the Amplatzer PMVSD occluder with encouraging initial results. This report presents initial and 1-year results from 54 patients with PMVSDs who underwent transcatheter closure at 5 different institutions with the Amplatzer PMVSD occluder. Sixty-five patients with PMVSDs were enrolled at 5 European centers. Eleven of the 65 patients did not fulfill the patient selection criteria at the initial echocardiographic evaluation or at cardiac catheterization. As a result, a total of 54 patients underwent attempted transcatheter closure using the Amplatzer PMVSD occluder. The median age of the patients was 5.1+/-3.6 years (range 0.3 to 13), and the median weight 18.5+/-10.3 kg (range 5 to 45). Devices were permanently implanted in 49 of 54 patients. Complete occlusion of the communication at 1-year follow-up was observed in 46 of 49 patients (94%). Main early procedural complications included (1) device embolization (2 patients), (2) severe bradycardia with hemodynamic compromise (2 patients), and (3) Mobitz II (2:1) heart block (1 patient). Late procedural complications included complete heart block (1 patient). No other complications were observed during follow-up. In conclusion, the Amplatzer PMVSD occluder is promising device that can be used for transcatheter closure in selected patients with PMVSDs. Further studies and long-term follow-up are required before this technique enters routine clinical practice.

Long-Term Neurodevelopmental Outcome and Exercise Capacity After Corrective Surgery for Tetralogy of Fallot or Ventricular Septal Defect in Infancy

Long-Term Neurodevelopmental Outcome and Exercise Capacity After Corrective Surgery for Tetralogy of Fallot or Ventricular Septal Defect in Infancy

Long-Term Neurodevelopmental Outcome and Exercise Capacity After Corrective Surgery for Tetralogy of Fallot or Ventricular Septal Defect in Infancy

Long-Term Neurodevelopmental Outcome and Exercise Capacity After Corrective Surgery for Tetralogy of Fallot or Ventricular Septal Defect in Infancy

Long-Term Neurodevelopmental Outcome and Exercise Capacity After Corrective Surgery for Tetralogy of Fallot or Ventricular Septal Defect in Infancy

The purpose of this prospective study was to assess whether neurodevelopmental status and exercise capacity of children 5 to 10 years after corrective surgery for tetralogy of Fallot or ventricular septal defect in infancy was different compared with normal children and influenced by the preoperative condition of hypoxemia or cardiac insufficiency. Forty unselected children, 20 with tetralogy of Fallot and hypoxemia and 20 with ventricular septal defect and cardiac insufficiency, operated on with combined deep hypothermic circulatory arrest and low flow cardiopulmonary bypass at a mean age of 0.7 +/- 0.3 years (mean +/- SD), underwent, at mean age 7.4 +/- 1.6 years, standardized evaluation of neurologic status, gross motor function, intelligence, academic achievement, language, and exercise capacity. Results were compared between the groups and related to preoperative, perioperative, and postoperative status and management. Rate of mild neurologic dysfunction was increased compared with normal children, but not different between the groups. Exercise capacity and socioeconomic status were not different compared with normal children and between the groups. Compared with the normal population, motor function, formal intelligence, academic achievement, and expressive and receptive language were significantly reduced (p < 0.01 to p < 0.001) in the whole group and in the subgroups, except for normal intelligence in ventricular septal defect patients. Motor dysfunction was significantly higher in the Fallot group compared with the ventricular septal defect group (p < 0.01) and correlated with neurologic dysfunction, lower intelligence, and reduced expressive language (p < 0.05 each). Reduced New York Heart Association functional class was correlated with lower exercise capacity and longer duration of cardiopulmonary bypass (p < 0.05 each). Reduced socioeconomic status significantly influenced dysfunction in formal intelligence (p < 0.01) and academic achievement (p < 0.05). Preoperative risk factors such as prenatal hypoxia, perinatal asphyxia, and preterm birth, factors of perioperative management such as cardiac arrest, lowest nasopharyngeal temperature, and age at surgery, and postoperative risk factors as postoperative cardiocirculatory insufficiency and duration of mechanical ventilation were not different between the groups and had no influence on outcome. Degree of hypoxemia in Fallot patients and degree of cardiac insufficiency in ventricular septal defect patients did not influence the outcome within the subgroups. Children with preoperative hypoxemia in infancy are at higher risk for motor dysfunction than children with cardiac insufficiency. Corrective surgery in infancy for tetralogy of Fallot or ventricular septal defect with combined circulatory arrest and low flow bypass is associated with reduced neurodevelopmental outcome, but not with reduced exercise capacity in childhood. In our experience, the general risk of long-term neurodevelopmental impairment is related to unfavorable effects of the global perioperative management. Socioeconomic status influences cognitive capabilities.

Need for closure of secundum atrial septal defect in infancy

Closure of isolated secundum atrial septal defect is generally recommended at the age of 4 to 5 years. However, there are children with isolated secundum atrial septal defect in whom early closure should be performed. We aimed to assess the underlying conditions that led to earlier closure in this special patient group and to analyze the outcome. From January 1990 through August 2002, 24 infants with isolated secundum atrial septal defect underwent surgical closure within the first year of life. All children were symptomatic. Signs of pulmonary hyperperfusion, such as tachydyspnea, failure to thrive, recurrent respiratory infections, or heart failure, were present. Four infants required artificial ventilation. Ten patients had additional problems, such as prematurity with chronic lung disease, hepato-omphalocele and congenital diaphragmatic hernia, which were present in 1 patient each. Eleven patients had defined dysmorphic syndromes. All but 1 infant underwent preoperative invasive hemodynamic evaluation. Thirteen patients had pulmonary hypertension preoperatively. The follow-up time was 46 +/- 33 months (range, 4-125 months). At follow-up, pulmonary artery pressure proved to be normal in 11 of the 13 children who had pulmonary hypertension previously. One patient died of persistent pulmonary hypertension. Clinical performance, growth, and development improved in nearly all patients. All ventilator-dependent children could be weaned shortly after atrial septal defect closure. If lungs are compromised, even a minor left-to-right shunt might be poorly tolerated in infancy. In these children early surgical closure of an isolated secundum atrial septal defect should be performed to support thrive and growth and to prevent the onset of irreversible changes of the pulmonary vasculature.

Atrial septal defect in infants & children

Atrial septal defect in infants & children

Usefulness of the mitral valve Z score in predicting the need to close moderate- to large-sized atrial septal defects in infants with aortic coarctation

Left ventricular anatomic substrates predisposing the development of congestive heart failure in infants with atrial left-to-right shunting after aortic coarctation repair were characterized. Mitral valve, but not aortic or left ventricular, hypoplasia correlated with the development of heart failure and the need for atrial defect closure shortly after coarctation repair.

Complete atrioventricular septal defect repair: Simplified single patch technique

Background: There has been a rekindling of interest in alternatives to conventional two patch technique for the repair of complete atrioventricular septal defect in infancy in the recent past. We applied the simplified single patch technique to 15 consecutive infants and herein report our intermediate term results. Methods: Between March 1998 and September 2001, fifteen patients underwent repair of complete atrioventricular septal defect with this technique (mean age 6 months, mean weight 5.4 kg). Downs syndrome was present in 11 patients. Repair was done in all patients by direct suturing of the common atrioventricular valve leaflets to the crest of the ventricular septum irrespective of the size of the ventricular septal component. The cleft in the anterior mitral leaflet was closed in all patients. The atrial septal component was closed by a pericardial patch. Results: There was no mortality. There were no pulmonary arterial hypertensive crises or heart block. The mean follow up was 13.2 months. One patient underwent mitral valve replacement after one year due to severe mitral regurgitation. The remaining fourteen patients had no significant mitral regurgitation, residual ventricular septal defect or left ventricular outflow tract obstruction on echocardiography. Conclusion: Simplified single patch technique is an easily reproducible method for surgical repair of complete atrioventricular septal defect. It is less time consuming and minimises ischaemic time. Atrioventricular valve function is preserved and there is no incidence of obstruction to left ventricular outflow tract. The intermediate term results are encouraging.

Correction of Complete Atrioventricular Canal

Correction of Complete Atrioventricular Canal

Clinical Analysis of Patch Repair of Ventricular Septal Defect in Infant

Background: Simple ventricular septal defect(VSD) is the most common congenital heart disease. Although closure of VSD is currently associated with a relatively low risk, experience with younger and smaller infants has been variably less satisfactory. We assessed the results of surgical closure of VSD in infant. Materials and Methods: Between 1996 and 2000, 45 non-restrictive VSD patients underwent patch repair and retrospective analysis was done. Patients were divided into two groups based on weight: group I infants weighed 5kg or less(n=16), and group II infants weighed more than 5kg(n=29). Both groups had similar variation in sex, VSD location, aortic cross clamp time and total bypass time. But combined diseases (ASD, PDA, MR) were more in group I. We closed VSD with patch and used simple continuous suture method in all patients. Results: There were no operative mortality, no reoperation for hemodynamically significant residual shunt and no surgically induced complete heart block. As a complication, pneumonia(group I: 2 cases, group II: 2 cases), transient seizure(group II: 2), wound infection(group I: 1, group II: 1), urinary tract infection(group I: 1) and chylopericardium(group I: 1) developed, and there was no significant difference between two groups(p>0.05). Conclusion: Early primary closure with simple continuous suture method was applicable in all patients with non-restrictive VSD without any serious complications.

Large ventricular septal defects in infants

Large ventricular septal defects account for important clinical problems during infancy, notably congestive heart failure. Timing of clinical presentation is dependent on the rapidity of decline in pulmonary vascular resistance. The basic physiologic abnormality is massive volume overloading of the pulmonary circulation and the left heart ( Q P/ Q S 3–4:1), For 1–3 months, pulmonary artery pressures are close to or at systemic levels, while the pulmonary vascular resistance is only slightly increased. Left-to-right shunting begins with isovolumic ventricular contraction and persists throughout systole, except for the period of isovolumic relaxation. Left-to-right shunting also takes place throughout diastole. The left ventricular pressure–volume curve is markedly increased in area, indicating an increase in stroke work. The inotropic state is difficult to define due to volume loading, but end-systolic pressure–volume studies indicate that there may be an element of decreased contractility in heart failure patients. Clinical signs and symptoms reflect volume loading of the pulmonary circulation, the development of systemic venous congestion and the operation of various adaptive mechanisms. Doppler echocardiography is particularly useful in localizing and sizing of the defect and in defining the flow dynamics. Cardiac catheterization and angiography is usually not required. Medical management primarily consists of the use of diuretics to decrease pulmonary and systemic venous congestion. The use of cardiac glycosides remains controversial. Afterload reduction with an angiotensin-converting enzyme inhibitor will diminish the left-to-right shunt, while enhancing systemic perfusion. Blood transfusion to elevate the hematocrit also diminishes shunting, while preserving oxygen transport. Early surgical correction is the preferred management after attempting to stabilize the clinical state by medical means. Delaying surgery is only indicated if there is documentation of the defect becoming smaller, favorable somatic growth and no evidence of an increase in pulmonary vascular resistance. Post-operative studies have demonstrated that with early surgical repair, normal growth and development occurs and left ventricular volumes and function normalize.

Closure of Isolated Secundum Atrial Septal Defects in Infancy

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.

Transcatheter closure of multiple muscular ventricular septal defects using Gianturco coils

A 10-month-old boy underwent operation to close a large secundum atrial septal defect and multiple muscular ventricular septal defects. Closure of the ventricular septal defects was unsuccessful and led to worsening cardiac failure and inability to wean the patient from mechanical ventilation. Transcatheter closure of the ventricular septal defects using Gianturco coils was undertaken. This technique is an effective alternative for closure of multiple muscular ventricular septal defects in infants and small children.