Ventricular Septal Defect Patients Research Articles

Right-heart vegetation penetrating through ventricular septal defect in patient with infective endocarditis

Right-heart vegetation penetrating through ventricular septal defect in patient with infective endocarditis

Unidirectional Monovalve Homologous Aortic Patch for Repair of Ventricular Septal Defect With Pulmonary Hypertension

Severe pulmonary hypertension is a common complication of congenital cardiac defects with large left to right shunt, and the closure of a large ventricular septal defect (VSD) with elevated pulmonary vascular resistance (PVR) is associated with significant morbidity and mortality. A unidirectional monovalve homologous aortic patch was designed to close the large VSD with severe pulmonary hypertension in an effort to decrease the morbidity and mortality. Twenty-seven patients (mean age, 15.0 +/- 5.6 years) with large VSD with severe pulmonary hypertension (pulmonary vascular resistance, 15.2 +/- 3.8 Wood units) were repaired with a unidirectional monovalve homologous aortic patch. According to body surface area and the preoperative arterial oxygen saturation, the monovalve homologous aortic patches were fenestrated on the aortic wall with a diameter of 4 to 8 mm. Two patients died of pulmonary hypertensive crisis and cardiac arrest postoperatively. All of the survival patients were followed up (5 months to 10 years) and the cardiopulmonary function was well improved with no late death. Obvious opening and closing of the monovalve was detected by early postoperative echocardiography in seven patients. A small amount of right to left shunt was detected in three patients three months after operation, and in two of them the shunt still existed three years after operation. Closure of a large VSD in patients with severe pulmonary hypertension could be performed with low morbidity and mortality when a unidirectional monovalve homologous aortic patch was used and the long-term result was satisfactory.

Down regulation of IGF-I and IGF-IR gene expression in right atria tissue of ventricular septal defect infants with right atria hypoxemia

Background Our previous studies showed serum insulin-like growth factor-I (IGF-I) concentrations significantly decreased in infants with congenital ventricular septal defect (VSD) and that they were associated with increased concentrations of growth hormone. In order to confirm the relationship between IGF-I axis and VSD, we further compared the IGF-I and insulin-like growth factor-I receptor(IGF-IR) gene expressions in the cardiac tissue of VSD infants. Methods Right atrium biopsies of 27 infants were studied. Five infants not having VSD were classified as controls (Group I). Twenty VSD patients were then divided into 2 groups according to their shunting magnitude index (level of pulmonary vascular resistance compared with systemic vascular resistance, Qp/Qs). VSD patients with minor shunts (Qp/Qs < 1.7) were classified as Group II; VSD patients with larger shunts (Qp/Qs ≥ 2) as Group III. Besides, seven tetralogy of fallot (TOF) with shunt (Qp/Qs > 4) infants were classified as the Group IV. A non-radioactive DIG-RNA probe detection system, western blotting and immunohistochemistry were used to detect the gene expression levels and protein products of IGF-I and IGF-IR in the right atrium samples of VSD infants. Results The relative protein levels of IGF-I were 0.96 ± 0.05, 0.43 ± 0.03, 0.15 ± 0.04, 0.12 ± 0.03 and IGF-IR were 0.80 ± 0.08, 0.57 ± 0.03, 0.38 ± 0.02, 0.24 ± 0.04 in the right atrium of 4 group patients. The relative mRNA levels of IGF-I were 0.95 ± 0.01, 0.41 ± 0.03, 0.29 ± 0.05, 0.15 ± 0.01 and IGF-IR were 0.85 ± 0.05, 0.56 ± 0.03, 0.17 ± 0.01, 0.18 ± 0.01, respectively. There was a significantly greater but more gradual decrease in protein levels and in mRNA levels of IGF-I and IGF-IR in Group II ( p < 0.05), Group III and IV ( p < 0.01) than in Group I. The results of immunohistochemistry also demonstrated a similar decrease in VSD patients. In addition, the decrease of mRNA and protein levels in IGF-I/ IGF-IR of VSD patients show related to the saturation of oxygen in the right atrium and the ratio of systolic right ventricular pressure to left ventricular pressure. Conclusion We further confirmed the down regulation of IGF-I/IGF-IR in cardiac tissue of VSD infants and the decrease to be associated with shunt magnitude and the severity of hypoxemia in the cardiac chamber of VSD.

Invited commentary

Invited commentary

Ventricular Septal Defect Closure in Right Coronary Cusp Prolapse and Aortic Regurgitation Complicating VSD in the Outlet Septum: Which Treatment is Most Appropriate?

There is currently not a standardized technique for the sizing and shaping of surgical closure of the ventricular septal defect (VSD) patch in patients with right coronary aortic cusp prolapse and aortic regurgitation (AR) complicating VSD in the outlet septum. Forty-six VSD patients who had aortic valve prolapse were divided into groups DC (direct closure, n=19), and SPC (small patch closure, n=27). Preoperative and postoperative echocardiography with Doppler color flow interrogation was performed on all patients. In the DC group, among seven patients who had aortic valve prolapse but no AR preoperative, one patient developed AR during postoperative follow-up period. In the remaining 12 patients who had mild AR associated with aortic valve prolapse prior to the procedure, AR was diminished in four and unchanged in six patients. However, AR was aggravated in two patients who required further operations for AV repair or replacement. In the SPC group, among the eight patients who had no preoperative AR, AR progressed in one patient postoperatively. In the remaining 19 patients who had mild AR, AR was diminished in 15 and unchanged in four. The outcome from the operative procedure was significantly better in the SPC group than DC group with mild preoperative AR (chi(2)=7.82; P<0.05). Small patch closure for this type of VSD is safer and more reliable in improving mild AR than that of direct closure, especially in patients with mild AR.

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.

Infective Endocarditis in Adolescents and Adults with Congenital Heart Disease

Background and Objectives:The development of new antibiotics and the advances in cardiac surgery has increased the number of patients with congenital heart disease (CHD) who grow into adulthood, and infective endocarditis (IE) has become one of the important complications. The aim of this study was to analyze the clinical findings of IE that occurred in adolescents and adults suffering with CHD. Subjects and Methods:In a retrospective case study, the medical records of 44 patients diagnosed as having IE were reviewed. The Duke criteria were used as the diagnostic criteria of IE. Results:Between October 1994 and December 2003, 44 patients and 49 cases were treated for IE with CHD in Samsung Medical Center. Their mean age was 45 years (median age: 44 years, range: 12-81 years) Ventricular septal defect (VSD) was the most common underlying cardiac disease (38.8%). In 18 of 49 cases (36.7%) CHD was diagnosed for the first time during the treatment of IE. 13 of the 31 patients with known CHD had not been seen on regular medical follow-ups. The common predisposing factors were dental procedures and acupuncture. The most common pathogens were Streptococcus viridans and Staphylococcus aureus. Valvular replacement was performed in 11 of the 19 VSD patients. Pulmonary complications developed in 12 (24.5%) and neurological complications developed in 10 (20.4%). The overall mortality was 2%. Conclusion: IE in adult CHD has become an important complication that infrequently accompanies a serious problem like severe valvular destruction or neurological complications. Proper management of CHD should be done early and educating the patient on the proper self-care for IE should be continued throughout life. (Korean Circulation J 2006;36:318-323)

Atrial and Ventricular Septal Defects Can Safely Be Closed by Percutaneous Intervention

Various transcatheter devices and methods to close congenital heart defects are currently available. Devices have been designed specifically for atrial septal defect (ASD), patent foramen ovale (PFO), and ventricular septal defect (VSD) closure. The trend in interventional treatment of intracardiac shunts shows toward defect-specific systems. The PFO is a tunnel defect requiring occluders that adapt to its length while common ASD strongly vary in their diameter, making a large scale of device sizes indispensable. VSDs are predominantly sealed by coils or tissue-adapted devices like muscular or perimembranous occluders. Since VSDs may occur with an aneurysm (VSA), a multi-perforated septum, an instable myocardial situation (postinfarction) or a high interventricular pressure gradient, closure of these defects is regarded sometimes as complicated. But during the last 30 years (since King and Mills implanted the first double-umbrella occluding system) several studies have proven procedure efficacy and safety of both ASD and VSD closure. This article reviews a large scale of studies and includes our single center data on 1,609 PFO, ASD, and VSD patients.

Primary transcatheter closure of postinfarction ventricular septal defects with the Amplatzer septal occluder- immediate results and up-to 5 years follow-up.

To report the periprocedural and long-term results of using the Amplatzer septal occluder for primary closure of post myocardial infarction ventricular septal defects. Transcatheter closure was considered in patients with significant left-to-right shunting and defect anatomy and location thought to be suitable for closure with such a device. From December 1999 until February 2005 eleven patients (9 males) aged 52-81 years (mean 67,9) underwent an attempted closure. The time from the onset of infarction to the procedure ranged between 2 days and 58 weeks (mean 15,4 weeks). There were three patients in an acute phase of infarction (three weeks or less). They were in critical condition and required inotropic and ventilatory support. Eight patients (all in a chronic infarction phase) were hemodynamically stable and in NYHA class III-IV (6 patients) or class II (2 patients). A successful device implantation occurred in all but one patient, in whom a 26 mm occluder pulled through a 16 mm defect on day 8 of infarction. An infarct exclusion surgery was successfully performed in this patient. In the remaining 10 patients, the defect size ranged 8-21 mm (mean 14,3), and the devices 11-30 mm (mean 19,3) were implanted. The procedure and screening time ranged 134-286 (mean 187,2) and 23-90 minutes (mean 43,6) respectively. The successful implantation did not clinically succeed in both patients with the acute septal rupture - they died 2 and 15 days after the procedure. In the eight patients in whom the procedure was performed late (3,5-56 weeks) after the infarction onset, the defect was either completely closed or the shunt was insignificant, and they improved dramatically. In the most recent follow-up from 1 to 62 months (mean 25,5), the patients have been alive and feeling well, and in NYHA I or II class. Primary transcatheter closure of postinfarction ventricular septal defects may be an alternative to surgery in patients with suitable anatomy and completed necrosis. In our experience, primary transcatheter closure of ventricular septal defects in patients who are in the acute phase of infarction does not improve their survival.

New Therapeutic Avenues with Hybrid Pediatric Cardiac Surgery.

Abstract Background: Minimally invasive strategies can be expanded by combining standard surgical and interventional techniques. Methods: A longitudinal prospective study was conducted of all pediatric patients who have undergone hybrid cardiac surgery at the University of Chicago Children's Hospital. Hybrid cardiac surgery was defined as combined catheter-based and surgical interventions in either one surgical setting or planned sequential surgical settings within a 24-hour period. Results: Between June 2000 and June 2003, 24 patients were treated with hybrid approaches. Sixteen patients with muscular ventricular septal defects (VSDs)with a mean age of 4 months (range, 2 weeks to 4 years) underwent either sequential Amplatzer device closure in the catheterization laboratory followed by surgical completion (group 1A [n = 9]: right ventricular (RV)outflow tract enlargement, 6 patients; closure of other VSDs, 5 patients; tricuspid valvuloplasty, 3 patients; bidirectional Glenn shunt, 1 patient; Maze procedure, 1 patient; and retrieval of embolized device, 1 patient) or, more recently, a 1-stage intraoperative off-pump device closure (group 1B;n =7)with the subsequent repair of concomitant heart lesions in 5 patients (double-outlet RV, 2 patients; arch hypoplasia/coarctation of the aorta, 2 patients; and pulmonary artery (PA) debanding, 1 patient). Cardioplegic arrest was either avoided or shortened in the muscular VSD patients. Eight patients with branch PA stenoses (group 2)underwent intraoperative PA stenting or stent balloon dilation along with RV outflow procedure (5 patients)or Fontan completion (3 patients with Maze procedure, mitral valvuloplasty, or Damus-Kaye-Stansel procedure in 1 patient each). All patients survived hospitalization. Complications from the hybrid approach in group 1A patients included tricuspid regurgitation in 2 patients, RV disk malposition in 1 patient, embolization of a VSD device into the aorta in 1 patient, and a residual VSD in 1 patient. No complications from the hybrid approach occurred in group 1B patients, and PA rupture from stent overinflation and ventricular dysfunction occurred in 1 patient each in group 2. During a mean follow-up period of 18 months (range, 2-36 months), 2 group 1A patients died suddenly several months after discharge. All of the other patients are doing well. Conclusions: Hybrid pediatric cardiac surgery performed in tandem by surgeons and cardiologists is a safe and effective means of reducing or eliminating cardiopulmonary bypass. Patients with muscular VSDs who are small, have poor vascular access, or have concomitant cardiac lesions are currently treated in one setting with the perventricular approach.

Staged repair of tetralogy of Fallot and diminutive pulmonary arteries with a fenestrated ventricular septal defect patch

Objectives Patients with tetralogy of Fallot and diminutive pulmonary arteries are at risk for suprasystemic right ventricular pressure and right ventricular failure after complete repair. We report the short-term outcome and medium-term follow-up after using a fenestrated ventricular septal defect patch as a component of staged repair in selected patients. Methods We reviewed 47 patients with tetralogy of Fallot and diminutive pulmonary arteries whose ventricular septal defect patch was fenestrated, either electively or as a rescue technique, at a single institution between 1984 and 2001. Results Early mortality was 10.6% and occurred only in patients who underwent rescue fenestration. Review of medium-term follow-up (median, 39 months) revealed 4 late deaths; an additional 4 patients experienced right ventricular failure despite fenestration. Most (7/8) of these late events occurred in patients who underwent planned fenestration. Excessive left-to-right shunt through the fenestration developed in only 2 patients. Conclusions Fenestrated patch closure of the ventricular septal defect in patients with tetralogy of Fallot and diminutive pulmonary arteries resulted in 10.6% early mortality. Used preemptively in selected patients, this technique is associated with no surgical mortality and a low incidence of excessive left-to-right shunt (4%). Early survivors remain at risk for late death and right ventricular failure despite fenestration.

Right ventricular collagen and fibronectin levels in patients with pulmonary atresia and ventricular septal defect.

Pulmonary atresia (PA) with ventricular septal defect (VSD) is an extreme form of tetralogy of Fallot with characteristic right ventricular hypertrophy. To reduce the right ventricular overload, these children have to undergo staged corrective surgery to restore physiological pulmonary perfusion. We studied the degree of fibrosis by analysing the myocardial expression pattern (at mRNA and protein level) of the extracellular matrix proteins, collagen and fibronectin in biopsies taken at corrective surgery from 14 patients affected by PA,VSD. Expression analysis by RT-PCR showed significantly higher levels for collagen III (p = 0.03), whereas collagen Ialpha (p = 0.31) and fibronectin (p = 0.47) mRNA levels remained unaltered in PA, VSD patients as compared to age matched controls. Video image analysis of immunohistochemical staining showed unchanged interstitial levels for total collagen (p = 0.17) as well as for fibronectin (p = 0.13) in the patients with PA, VSD. However, peri-vascular staining for collagen (p < 0.01) and fibronectin (p = 0.02) represented as the peri-vascular stained area corrected for the vessel lumen area showed significantly decreased levels in the PA, VSD group as compared to controls. Our results indicate that the patients with PA, VSD have inadequate extracellular matrix support for their coronary blood vessels and perhaps due to an altered biosynthesis of collagen and fibronectin network.

Full Sternotomy with Minimal Skin Incision for Congenital Heart Surgery

Purpose: The purpose of this paper is to analyze the feasibility of the full sternotomy with minimal skin incision and its related complications and risks. Methods: A total of 405 patients with simple congenital heart disease underwent open heart surgery exclusively under full sternotomy with minimal skin incision. We reviewed the available medical records of the patients retrospectively. Bypass time, aorta cross clamp time, and period of hospital stay were compared with the control group (223 patients with standard long skin incision). Results: Full sternotomy with minimal skin incision provided adequate surgical views and successful repair was done in all patients. There was no mortality. One patient had chylopericardium after the operation and another patient had a postoperative bleeding at the sternum. Minimal skin incision took the similar aorta cross-clamp time and total cardiopulmonary bypass time compared with full skin incision in atrial septal defect patients. Among the ventricular septal defect patients, minimal skin incision took a little longer aorta cross-clamp time (10%). but similar total cardiopulmonary bypass time compared with full skin incision. Conclusion: Minimal skin incision with full sternotomy provides improved cosmetic results. There was no increased mortality and morbidity using minimal access. It can be applied to more complex congenital heart disease contrast to other minimal invasive techniques for atrial septal defect.

Full sternotomy with minimal skin incision for congenital heart surgery

Purpose: The purpose of this paper is to analyze the feasibility of the full sternotomy with minimal skin incision and its related complications and risks. Methods: A total of 405 patients with simple congenital heart disease underwent open heart surgery exclusively under full sternotomy with minimal skin incision. We reviewed the available medical records of the patients retrospectively. Bypass time, aorta cross clamp time, and period of hospital stay were compared with the control group (223 patients with standard long skin incision). Results: Full sternotomy with minimal skin incision provided adequate surgical views and successful repair was done in all patients. There was no mortality. One patient had chylopericardium after the operation and another patient had a postoperative bleeding at the sternum. Minimal skin incision took the similar aorta cross-clamp time and total cardiopulmonary bypass time compared with full skin incision in atrial septal defect patients. Among the ventricular septal defect patients, minimal skin incision took a little longer aorta cross-clamp time (10%), but similar total cardiopulmonary bypass time compared with full skin incision. Conclusion: Minimal skin incision with full sternotomy provides improved cosmetic results. There was no increased mortality and morbidity using minimal access. It can be applied to more complex congenital heart disease contrast to other minimal invasive techniques for atrial septal defect.

Heart–lung or lung transplantation for Eisenmenger syndrome

Background: The optimal therapy for end-stage Eisenmenger syndrome (ES) is unknown. We analyzed the United Network for Organ Sharing/International Society for Heart and Lung Transplantation Joint Thoracic Registry to determine predictors of survival. Methods: Univariate analysis was performed using Kaplan–Meier survival curves. Groups were compared using the log-rank test. Multivariate analysis was performed using a proportional hazards model. Results: There were 605 transplants performed between 1988 and 1998. The causes of ES included atrial septal defect (ASD) in 171, ventricular septal defect (VSD) in 164, multiple congenital anomalies (MCA) in 68 and patent ductus arteriosus (PDA) in 32. Procedures included 430 heart–lung (HLT), 106 bilateral lung, and 69 single lung transplants (LT). Survival after HLT was better than after LT on univariate analysis ( p = 0.002). For HLT, survival at 30 days and 1 year was 80.7% and 70.1% compared with 68% and 55.2% for LT. Diagnosis was also a significant predictor of survival ( p = 0.011), being best for VSD and MCA (1-year survival 71.4% and 77.6%). There was a highly significant benefit of HLT over LT for VSD patients ( p = 0.0001). Diagnosis, the combination of diagnosis and procedure, recipient age, recipient gender, donor age, ischemic time and recipient status were significant in a multivariate model. Multivariate analysis confirmed the superior prognosis of patients with VSD or MCA ( p = 0.007 and p = 0.022, respectively) and suggested that the adverse effect of LT was predominately in patients with VSD (risk ratio 1.817, p = 0.035). Conclusions: This analysis suggests that ES recipients are not a homogeneous group. Patients with VSD and MCA have the best prognosis. HLT appears to offer a survival benefit for patients with ES secondary to VSD and should be re-considered as the operation of choice.

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.

Long-term results of relief of subaortic stenosis in univentricular atrioventricular connection with discordant ventriculoarterial connections

Background. We set out to examine the long-term results of relief of subaortic stenosis by enlargement of ventricular septal defect in patients with univentricular atrioventricular connection to a dominant left ventricle and discordant ventriculoarterial connections. Methods. Twenty-four patients underwent enlargement of ventricular septal defect between 1985 and 1998 at a median age of 3.2 years (range, 3 weeks to 14 years). Ten patients were younger than 1 year of age. Eighteen had undergone previous banding of the pulmonary trunk, 9 of whom also required repair of coarctation of the aorta. The median subaortic gradient before enlargement was 46 mm Hg. Twenty-three patients had a patch to enlarge the rudimentary right ventricle. Results. Five patients (21%) died in the early postoperative period. The overall survival at 1 and 3 years was 73%, and at 5 and 10 years was 68% and 60%, respectively. Complete heart block requiring insertion of a pacemaker occurred in 2 patients (8%). A Fontan operation was performed in 10 patients, 5 underwent a bidirectional Glenn procedure, and 2 required cardiac transplantation. Follow-up was complete in all survivors at a median time of 6.7 years (range, 8 months to 13 years). From the earlier part of the series, 3 patients experienced aortic insufficiency and 2 had recurrent obstruction. Factors adversely affecting survival were age younger than 1 year at operation and presence of obstruction within the aortic arch. Conclusions. Our experience shows that, in patients with univentricular atrioventricular connection to a dominant left ventricle and subaortic stenosis, enlargement of the ventricular septal defect provides satisfactory relief of obstruction except in those younger than 1 year of age, and those who have associated obstruction in the aortic arch.

Pulmonary arterial compliance in children with atrial and ventricular septal defect

Previous studies have documented the normal values of pulmonary arterial compliance (Cp) in animals and adult humans. In the past, variations in Cp and its measurement in children with septal defects has been unknown. In the present study, we found the Windkessel model, which uses compliance and resistance as parameters, to be a useful tool in understanding Cp in pediatric patients. Calculations of Cp were based on the pulmonary arterial diastolic pressure waveform as an exponential function of time. First, Cp was estimated by studying pressure tracings of the main pulmonary artery (MPA) obtained from both routine cardiac catheterization and pressure measured by a catheter-tip manometer, which was performed during a catheterization study of seven children with various congenital heart diseases. Second, 124 children with atrial and ventricular septal defects aged between 45 days and 12 years were studied using the data obtained from routine catheterization. Hemodynamic data were used to calculate pulmonary vascular resistance (Rp), pulmonary arterial time constant (Tp) and Cp. A strong correlation (r = 0.954) was found in the Cp value estimated by data obtained from routine catheterization and from the catheter-tip manometer study. The present study shows an estimated mean (SEM) Cp in normal children of 1.53 (0.17) ml/mmHg per m2. The estimated mean (SEM) Cp was 1.91 (0.10) and 1.70 (0.11) ml/mmHg per m2 in children with atrial septal defect (ASD) and ventricular septal defect (VSD), respectively. It was found that Cp was significantly (P = 0.04) higher in female patients with a VSD. Also, a significantly low Cp (0.95 +/- 0.06 ml/mmHg per m2) was observed in patients with a VSD and pulmonary hypertension (VSDPH). In conclusion, Cp was calculated by the exponential decay portion of the MPA diastolic pressure waveform. A normal Cp value was observed in ASD and VSD patients and a significantly low Cp was observed in children with a VSDPH. Cp was higher in female VSD patients than in male VSD patients.

Congenital obstructive lesions of the right aortic arch

Background. In the setting of normal cardiac situs, a right-sided aortic arch is uncommon. When a right arch does occur, it is typically in conjunction with other congenital cardiovascular anomalies, especially defects with abnormal right ventricular outflow. Congenital obstruction of a right arch, caused by coarctation, interruption, or cervical arch, is extremely rare.Methods. We reviewed our experience and all reported cases of right aortic arch with coarctation of the aorta, interrupted arch, or obstruction of a cervical arch in the setting of normal cardiac situs and topology.Results. Since 1992, 4 such patients have undergone repair at our institution, including 1 with interrupted arch, 1 with coarctation of a mirror image arch, and 2 with obstruction of a cervical arch. In addition to these 4 patients, 38 others have been described in the published reports: 15 with interrupted arch, 19 with coarctation, and 4 with obstruction of a cervical arch. Associated cardiac defects were uncommon, except for ventricular septal defect in patients with interrupted arch, but abnormalities of the brachiocephalic vessels were frequent. Except for most of the patients with interrupted right arch, the majority of patients described have undergone successful surgical repair.Conclusions. Although obstructive arch lesions are often grouped together, the etiologies of coarctation of the aorta, interrupted arch, and cervical arch with obstruction almost certainly differ. The rarity of such lesions among patients with right aortic arch may be explained in part by the fact that the fetal hemodynamic conditions associated with persistence of a right arch do not facilitate flow-related arch obstruction. In this review, we discuss these issues in detail, along with specific surgical considerations in the management of obstruction lesions of the right aortic arch.

Relation between plasma nitrate and mean pulmonary arterial pressure in ventricular septal defect

BACKGROUNDNitric oxide (NO) is known to modulate myocardial contraction and coronary tone, and its inhalation reduces pulmonary vascular resistance in patients with pulmonary hypertension.OBJECTIVESTo evaluate the pathophysiological role of NO...