The early and late effects of surgical closure of ventricular septal defects on pulmonary vascular dynamics

In sixty patients who underwent closure of ventricular septal defect (VSD), cardiac catheterizations were performed before and late after surgery together with pressure measurements immediately after closure of the VSD during surgery. Pulmonary arterial mean pressure (PAm), pulmonary arterial systolic pressure to systemic arterial systolic pressure ratio (Pp/Ps), and pulmonary vascular resistance to systemic vascular resistance ratio (Rp/Rs) were measured and calculated. The patients were classified into 5 groups according to the preoperative Rp/Rs and Qp/Qs as was reported by Nakada: Group A: Rp/Rs less than 0.15, Qp/Qs larger than or equal to 1.8, Group B: Rp/Rs less than 0.15, Qp/Qs less than 1.8, Group C: 0.15 less than or equal to Rp/Rs less than 0.50, Group D: 0.50 less than or equal to Rp/Rs less than 0.85, Group E: 0.85 less than or equal to Rp/Rs. These groups were further divided into 3 groups respectively according to age at operation (less than or equal to 2 years, 3 or 4 years, 5 years less than or equal to). The averages of PAm, Pp/Ps, and Rp/Rs were within the normal range in Group A and B patients (normal pulmonary vascular resistance groups) irrespective to the age at operation except the average of PAm before surgery. In Group C, D and E patients (elevated pulmonary vascular resistance group), these variables decreased immediately after closure of VSD, and further decreases were noted at the time of late catheterization. These variables, however, did not completely normalize even at that time. Among the patients operated upon at 2 years of age or less, the averages of these variables normalized immediately after closure of the VSD. When operated upon at 3 or 4 years of age, these variables decreased but did not normalize immediately after closure of the VSD and were found to be within the normal range at the time of late catheterization. When operated upon at 5 years of age or more, these variables decreased immediately after closure of the VSD, and further decrease was found at the time of late catheterization but mostly remained in the abnormal range even at this time. From the data obtained herein, the factors producing the pulmonary vascular resistances in respective age groups were discussed. The closure of VSD in patients with elevated pulmonary vascular resistance is recommended at the latest 4 years of age and preferably at 2 years of age or less, in order to obtain normal pulmonary circulatory dynamics after surgery.     

Blood grouping problems

From 1965 to 1974, 53 children with coarctation of the aorta (COA) and an associated ventricular septal defect (VSD) underwent cardiac catheterization. Thirty-one patients presented with congestive heart failure. Twenty-five of 27 patients (92%) who underwent cardiac catheterization under age 3 months had either systemic hypertension, a systolic gradient across the coarctation greater than 20 mm Hg or both. Pulmonary hypertension was present in all 25 patients. COA repair was performed in 39 patients and there were seven deaths. Of the 32 survivors, 23 have no residual gradient; six are normotensive but have a mild residual gradient; three are hypertensive or have a gradient greater than 20 mm Hg. Repair of the VSD or pulmonary artery banding has been performed in 11 of 44 patients who survived infancy. Spontaneous closure of the VSD has occurred in three cases and 25 patients have a small VSD that does not warrant surgical repair. Surgical repair of COA during infancy may be unavoidable but conservative medical management of the associated VSD is often successful.     

Double patch closure of ventricular septal defect with increased pulmonary vascular resistance

BACKGROUND: Closure of a large ventricular septal defect (VSD) in children with elevated pulmonary vascular resistance is associated with significant morbidity and mortality. Pulmonary hypertensive episodes continue to be a major cause of postoperative morbidity and mortality. We designed a fenestrated flap valve double VSD patch in an effort to decrease the morbidity and mortality associated with the closure of a large VSD with elevated pulmonary vascular resistance. METHODS: Eighteen children (mean age, 5.7 years) with a large VSD and elevated pulmonary vascular resistance (mean, 11.4 Wood units) underwent double patch VSD closure using moderately hypothermic cardiopulmonary bypass and cardioplegic arrest. The routine VSD patch was fenestrated (4 to 6 mm) and on the left ventricular side of the patch, a second, smaller patch was attached to the fenestration along its superior margin before closure of the VSD. RESULTS: All children survived operation and were weaned from inotropic and ventilator support within 48 hours postoperatively. Postoperative pulmonary artery pressures were significantly lower than preoperative values. One child died 9 months postoperatively. CONCLUSIONS: Closure of a large VSD in children with elevated pulmonary vascular resistance can be performed with low morbidity and mortality when a flap valve double VSD patch is used.     

Bidirectional shunt flow across a ventricular septal defect: pulsed Doppler echocardiographic analysis

Pulsed Doppler echocardiographic and hemodynamic examinations were performed in 31 patients (mean age 17.8 years) with isolated ventricular septal defect (VSD). Three groups were studied: group I (n = 6) patients had severe pulmonary vascular obstructive disease (PVOD); group II (n = 12) patients had pulmonary hypertension (PH) without severe PVOD; group III (n = 13) patients had no PH. Bidirectional shunting was detected in 9 VSD patients (6 in group I and 3 in group II). Patients with low to moderately elevated right ventricular pressures demonstrated left-to-right shunting across the defect throughout the cardiac cycle. When systolic pressure in the right ventricle reached approximately 60% of the left ventricular pressure, right-to-left shunting occurred across the defect during early and mid diastole. However, in patients with Eisenmenger syndrome (group I) the right-to-left shunting occurred during late systole with continuation during the early and mid diastolic period. The earlier occurrence of right-to-left shunting (index < 0.5 second) signifies the presence of severe PVOD.     

Development of double-chambered right ventricle after surgical closure of a ventricular septal defect

The double-chambered right ventricle (DCRV) is a rare cardiac malformation, often associated with ventricular septal defect (VSD). CASE REPORT: The authors describe a case of DCRV in a child who underwent surgical repair of perimembranous VSD at the age of 7 months because of systemic pulmonary artery hypertension and growth retardation. At this time, no pressure gradient was measured within the right ventricle and no muscle proliferation was observed on echocardiography or right ventriculography. Postoperative follow-up revealed hypertrophy of the moderator band accompanied by progressive development of a right midventricular pressure gradient, which reached 60 mm Hg at the age of 3 1/2 years. The child was operated after invasive confirmation of the diagnosis of DCRV and the haemodynamic data. The child is now 6 years old and no restenosis has been observed. CONCLUSION: DCRV is usually associated with VSD, but the course of the two diseases can be dissociated. The diagnosis of DCRV must be considered in any child with a history of VSD, even after surgical or spontaneous closure, when clinical examination reveals a systolic ejection murmur.     

Controllable graded cerebral ischaemia in the gerbil: studies of cerebral blood flow and energy metabolism by hydrogen clearance and 31P NMR spectroscopy

Patent ductus arteriosus is an uncommon anomaly in adult patients. Surgical closure of patent ductus arteriosus in this age group presents difficult problems to the surgeon. We report our experience of 21 adult patients (19-62 years of age, mean 40 years) who underwent closure of the ductus by transfemoral implantation of a Rashkind double umbrella device. The patients came to light because of atrial fibrillation, congestive heart failure, residual flow after surgical ligation of the duct or because of incidental diagnosis made during physical examination or chest X-ray. In ten patients the pulmonary arterial pressure was normal (systolic pressure < 30 mmHg), in eleven it was elevated (systolic pressure from 30 to 100 mmHg, mean 50 mmHg). In seven patients the duct was clearly calcified and the size of the duct varied from 3 to 9 mm (mean 4.3 mm). In 16 patients the ductus resulted perfectly closed after implantation of the first double umbrella device, two patients had minimal residual aortopulmonary flow, whereas in three patients the residual shunt was significant; two of these also developed haemolysis and went to surgery, in the latter the shunt was completely abolished after implantation of a second 17-mm device 16 months later. In conclusion transcatheter closure of patent ductus arteriosus in adults is feasible, even in the presence of calcifications and/or pulmonary hypertension; taking into account the significant surgical risk, PDA umbrella closure should be considered the first choice procedure in this group of patients.     

Ileocecal tuberculosis as a cause of lower gastrointestinal hemorrhage in lupus erythematosus

BACKGROUND AND AIMS OF THE STUDY: The use of living, untreated autologous pericardium for patch repair in the left ventricular outflow tract was considered attractive in children. METHODS: Ventricular septal defect (VSD) closure with an untreated autologous pericardial patch was performed in 102 children of mean age 13.4 months (range: 1 to 73 months). Postoperative transthoracic Doppler echocardiography was performed in all children at a mean of nine weeks (range: one day to 50 weeks) after surgery. One pericardial patch, which was explanted at autopsy two months after surgery, was studied microscopically. RESULTS: At short-term follow up, no or only minor residual VSD was found in 97 patients, moderate VSD in two and severe VSD in one patient. One patient was reoperated for residual VSD and an aneurysmic patch first diagnosed seven days after surgery. Two more patients showed ballooning of the patch without VSD after five and seven days respectively. All aneurysmic patches were attributed to intraoperative patch oversizing. Patch integrity was confirmed in all other patients. No inflammatory or degenerative changes were observed at microscopy, rather a remodeling response had caused the patch to thicken, indicating an adaptation of the living tissue. CONCLUSIONS: The untreated autologous pericardial patch has shown to be a safe alternative for VSD closure, provided that the patch is properly sized.     

Pediatric lung transplantation. Indications, techniques, and early results

From July 1990 to April 1993, 36 lung transplantations in 33 patients were performed in our pediatric transplant program (0.25 to 23 years, mean age 10.3 years). Eight children had been continuously supported with a ventilator for 3 days to 4.5 years before transplantation and three were supported by extracorporeal membrane oxygenation. Indications for lung transplantation in this pediatric population included the following: cystic fibrosis (n = 13), pulmonary hypertension, and associated congenital heart disease (n = 10), pulmonary atresia, ventricular septal defect and nonconfluent pulmonary arteries (n = 3), pulmonary fibrosis (n = 6), and acute respiratory distress syndrome (n = 1). Three children underwent retransplantation for acute graft failure (n = 2) or chronic rejection (n = 1). Pulmonary fibrosis was related to complications of treatment of acute of myelogenous leukemia with bone marrow transplantation in two children and to bronchiolitis obliterans, bronchopulmonary dysplasia, interstitial pneumonitis, and Langerhans cell histiocytosis in four others. Thirteen children underwent lung transplantation and concomitant cardiac repair. Bilateral lung transplantation, ventricular septal defect closure and pulmonary homograft reconstruction of the right ventricular outflow tract to the transplanted lungs was performed in three children by means of a new technique that avoids the need for combined heart-lung transplantation. Two patients had ventricular septal defect closure and single lung transplant for Eisenmenger's syndrome, two had ligation of a patent ductus arteriosus and transplantation, three additional children underwent atrial septal defect closure and lung transplantation, and two underwent lung transplantation for congenital pulmonary vein stenosis. Eight early deaths and three late deaths occurred (actuarial 1-year survival 62%). Lung transplantation in children has been associated with acceptable early results, although modification of the adult implantation technique has been necessary. Lung transplantation and repair of complex congenital heart defects is possible; heart-lung transplantation may only be required for patients with severe left heart dysfunction and associated pulmonary vascular disease. Bronchiolitis obliterans remains a major concern for long-term graft function in pediatric lung transplant recipients.     

Quantitative analysis of pulmonary vascular disease in complete transposition of the great arteries

Forty autopsy cases of complete transposition of the great arteries (TGA) and 22 autopsy cases of ventricular septal defect (VSD) were analyzed histologically for evidence of vascular damage due to pulmonary vascular disease (PVD). Positive correlations were generally observed between an index of pulmonary vascular disease (IPVD) and blood pressure of pulmonary circulation. No significant difference in IPVD was found between TGA and VSD in the first five months of life, when cases of each disease were compared at similar blood pressure levels. After that age, however, IPVD was much higher in TGA, and particularly severe PVD in this disease was demonstrated histologically. Morphometrical analysis of the pulmonary artery revealed hypertrophy of the muscular cost in response to elevated blood pressure. However, the progress of medial hypertrophy was retarded in TGA in the first five months, and medial thickness in arteries of cases of TGA older than five months was only 70% of that in VSD at the same blood pressure levels. Suppression of this process of reinforcement of the arterial wall in response to the stress of high pulmonary pressure was regarded as one of the important factors precipitating severe pulmonary vascular disease in transposition of the great arteries.     

Repair of ventricular septal defect in the first two years of life using profound hypothermia-circulatory arrest techniques

Ventricular septal defect repair had been performed in 57 infants ages 21 days to 21 months and under 10 kg in weight using profound hypothermia-circulatory arrest technics. Severe congestive heart failure was the indication for operation in all but two infants under 6 months of age, and in those under 3 months there was usually an associated moderate or large sized atrial septal defect or patent ductus arteriosus or a coarctation. In infants over 6 months controlled heart failure was accompanied by failure to thrive and often recurrent respiratory infections. The main indication for surgery in three infants was repeated severe respiratory infections and in 7 infants, ages 10-15 months, an elevation of pulmonary vascular resistance of 6 units M2 or more. There were two hospital deaths among the 49 infants without coarctation (ages 6 and 20 months) and two among the 8 with coarctation. Postoperative respiratory and other complications were uncommon. On late review there was no significant residual VSD amongst the 11 recatheterized patients. Psychometric studies in 19 children who had reached the age of three to four years gave no evidence of cerebral damage due to the circulatory arrest period. In view of these results palliative pulmonary artery banding is no longer performed for VSD in infancy unless there is a Swiss cheese septum or an associated severe coarctation.     

Unidirectional valve patch for repair of cardiac septal defects with pulmonary hypertension

BACKGROUND: Congenital septal defects with a large left-to-right shunt often cause pulmonary hypertension, which complicates surgical repair of the defects. METHODS: Twenty-four patients with congenital cardiac septal defects and severe pulmonary hypertension had operation to close the septal defect using a unidirectional valve patch during a 3-year period. The ratio of systolic pulmonary artery pressure to systolic arterial blood pressure was near to or more than 1.0 in all patients. RESULTS: Two patients died in the hospital after operation, and there have been no deaths during intermediate term follow-up. Mean pulmonary artery pressure decreased from 80 +/- 12 mm Hg to 56 +/- 18 mm Hg. The ratio of pulmonary artery pressure to systemic arterial pressure dropped from 1.1 +/- 0.1 mm Hg to 0.7 +/- 0.1 mm Hg. The unidirectional valve patch functioned allowing right to left shunting in 4 patients with a systolic pulmonary artery pressure more than systolic arterial blood pressure immediately after closure of a septal defect. The patch sealed or was effectively closed by the third postoperative day. There was impressive improvement in symptoms and exercise tolerance after operation during the 3-month to 3-year (mean, 1.1 year) follow-up period. CONCLUSIONS: The unidirectional valve patch is useful for management of patients having operation to close cardiac septal defects in the presence of severe pulmonary hypertension.     

Brain natriuretic peptide is stable in whole blood and can be measured using a simple rapid assay: implications for clinical practice

Patients with moderate and severe pulmonary hypertension have a very high mortality rate when undergoing orthotopic liver transplantation. Because nitric oxide has been successful in reducing pulmonary artery pressures in certain patients with pulmonary hypertension, the efficacy of NO inhalation (40 and 80 ppm) in 4 patients with pulmonary hypertension associated with liver disease was determined. No clinically significant changes in pulmonary artery pressures or other hemodynamic parameters were observed using either concentration of NO. In conclusion, no pulmonary vasodilatory response from inhalation of NO in 4 patients with severe liver disease and pulmonary hypertension was found.     

Fibrosing mediastinitis causing pulmonary arterial hypertension without pulmonary venous hypertension. Clinical and necropsy observations

Clinical and morphologic observations are described in two patients with severe pulmonary arterial hypertension without pulmonary venous hypertension from fibrosing mediastinitis. In one patient, both main pulmonary arteries and one major pulmonary vein were severely narrowed by dense fibrous tissue; in the second patient, only the right main pulmonary artery was severely narrowed. Both patients had normal intrapulmonary arteries and normal pulmonary parenchyma. Of nine previously described necropsy patients with pulmonary hypertension due to fibrosing mediastinitis, seven had severe narrowing of multiple large pulmonary veins and in six of them the pulmonary hypertension was entirely due to pulmonary venous obstruction. In one other patient, the pulmonary hypertension was due to obstruction of one main pulmonary artery and several large pulmonary veins. Each of these seven previously described patients had severe changes in the small intrapulmonary arteries. Of the other two previously described patients with pulmonary hypertension from fibrosing mediastinitis, one had severe narrowing of only the main right pulmonary artery, and the other, of both main pulmonary arteries. Thus, although pulmonary arterial hypertension in patients with fibrosing mediastinitis is usually due to obstruction of multiple large pulmonary veins and to severe secondary changes in small intrapulmonary arteries, fibrosing mediastinitis can cause severe pulmonary hypertension by obstructing the right or both main pulmonary arteries.     

Effects of low doses of ionizing radiation and induced hypothyroidism on the course of pregnancy and the development of the offspring in rats

OBJECTIVES: To determine the appropriate indications and timing for surgery in children with either a perimembranous or a subarterial type of ventricular septal defect (VSD) associated with aortic cusp prolapse. DESIGN: Retrospective review of children with VSD and associated aortic cusp prolapse with or without aortic regurgitation. This review was based on data obtained from clinical findings, two-dimensional echocardiography, cardiac catheterization and angiocardiography. SETTING: Tertiary health care facility with two-dimensional and colour Doppler echocardiographic and cardiac surgery facilities, and a catheterization laboratory. PATIENTS: Forty-eight patients were found to have perimembranous or subarterial VSDs in association with aortic cusp prolapse with or without aortic regurgitation. INTERVENTIONS: All 48 patients had high resolution two-dimensional and colour Doppler echocardiography. Of the 19 patients who underwent surgical closure of their VSD, five also had an aortic valvuloplasty and one had an aortic valve replacement. Cardiac catheterization was performed in 16 of the 19 surgical patients and 12 of the 29 nonsurgical patients. MEASUREMENTS AND MAIN RESULTS: Annual clinical and echocardiographic assessments in the nonsurgical group did not demonstrate increasing aortic insufficiency. Two children in the nonsurgical group showed spontaneous resolution of aortic insufficiency. In the surgical group, four children with VSD and clinical aortic insufficiency had surgery at less than five years of age; two were found to be regurgitant-free, one had trivial clinical aortic insufficiency and the other had echocardiography-only insufficiency. Of the seven surgical patients older than five years with VSD and clinical aortic insufficiency, four were found to be regurgitant-free, one had echocardiography-only regurgitation and two were unchanged. Two children undergoing surgery with VSD and no aortic insufficiency had postoperative echocardiography-only regurgitation, presumably related to cusp deformity from presurgical prolapse. Children with large VSDs with or without aortic cusp prolapse required surgery for indications of shunt size and pulmonary resistance. CONCLUSIONS: For children with small perimembranous VSDs and cusp prolapse, surgery is indicated only if there is clinical evidence of aortic regurgitation and progressive left ventricular enlargement.     

Ligation of medically refracted patent ductus arteriosus (PDA) in an extremely low body weight premature infant

Medically refracted patent ductus arteriosus (PDA) in an extremely low birth weight (ELBW) preterm (gestation 24 weeks 2 days) infant was successfully ligated under general anesthesia in the neonatal intensive care unit (NICU). Pharmacological agents are more effective to close PDA in preterm infants than in full-term infants, although within 48 h three doses of indomethacin were not sufficient to close PDA in this case. At the age of 69 h the infant developed severe symptoms including bradycardia, systemic hypotension, pulmonary hypertension, diastolic steal (reverse distal aorta flow velocity), and anuria. A PDA ligation was thus performed surgically at 72 h of age. General anesthesia and surgical stress were tolerated by this 531 g infant. Postoperatively all symptoms improved dramatically and the general conditions were stable. On the 38th day the endotracheal tube was extubated and on the 50th day nasogastric milk feeding was started. The oxygen supply was weaned on the 78th day. Growth and development until 6 months were within the normal range of very low birth weight infants. A surgical ligation as early as possible in medically refracted PDA in an ELBW infant is thus considered to be a safe and effective treatment. It prevents the development of further complications of cardiopulmonary vascular problems. Color Doppler echocardiography can reliably measure the PDA size, flow velocity, and hemodynamic changes of persistent PDA, even in tiny infants.     

Percutaneous closure of patent ductus arteriosus using special screwing detachable coils

Jackson screwing detachable coils, developed for arterial and venous embolization, have been successfully employed recently for the percutaneous occlusion of patent ductus arteriosus (PDA). Special screwing detachable coils were designed for closure of the PDA, and the experience gained by their use is described in this report. Occlusion was attempted in 29 patients with a minimal ductal diameter of 0.9-4.2 mm. Coils were successfully placed in all 29 patients. One coil, which embolized 10 min after detachment, was retrieved. In 26 patients (89%), complete closure was achieved. Only 35 coils were placed in 29 patients. Residual shunts in three patients are minimal, detectable only on color-flow mapping. Screwing detachable coils for closure of PDA are safe and effective for occlusion of PDA with a minimal diameter < 4.2 mm. Embolization of the coil is very rare. A high closure rate is achieved with a low number of placed coils.     

Transcatheter closure of patent ductus arteriosus using controlled-release coils

Controlled-release coils have become available recently for the closure of patent ductus arteriosus (PDA). Transcatheter closure of patent arterial ducts was attempted in 13 patients, ranging in age from 5 months to 15 years, mean 4.1 years. Implantation of controlled-release PDA coils was attempted via the femoral artery through 5 Fr catheters in all cases except one, in whom both the femoral arterial and venous routes were used. The procedure was successful in 10 of the 13 patients. In these, the pulmonary artery systolic pressure ranged between 25 and 42 mmHg and the duct diameter varied from 1.5 to 6 mm at its narrowest point. Six of the patients received a single coil. Two coils were inserted in three patients and three coils in one patient. In three patients the ducts were too large for safe release of the coils, despite attempted implantation of up to three coils simultaneously. These coils were easily withdrawn into the catheter. Immediately at the end of the procedure, the duct was completely occluded in nine of the 10 patients, and in one patient there was a small residual flow. The procedure time varied between 35 min and 2.5 h, mean 81 min and the fluoroscopy time varied from 5 to 78 min, mean 25 min. None of the patients experienced hemorrhage, diminished lower extremity pulse, hemolysis or infection. In one patient, a 5 mm coil embolized into the right pulmonary artery soon after release. It was retrieved with a snare, then 8 mm and a 5 mm coil were implanted satisfactorily in the arterial duct. At follow-up by color Doppler echocardiography, the duct was completely occluded in all patients. Transcatheter closure of patent arterial ducts by controlled-release PDA coils is effective and safe. Even when more than one coil is inserted, it is still cheaper than transcatheter umbrella closure. This method is therefore of great value, particularly in less affluent countries.     

Primary pulmonary hypertension in pregnancy: a case report

Primary pulmonary hypertension is a rare, incurable, and progressive disease. When it is associated with pregnancy, the mortality rises to > 50%. We report a patient who was diagnosed with severe pulmonary hypertension of unknown cause in the twenty-second week of gestation. She was treated with an oral calcium-channel blocker and low-molecular-weight heparin and underwent delivery by cesarean section with good maternal and fetal outcome. Patients with severe pulmonary hypertension should, however, avoid pregnancy because of the high mortality, although cases have been reported with positive outcome.     

Histologic specimen collection in pulmonary hypertension using percutaneous, transvascular biopsy--animal experiment and initial clinical data]

Pulmonary hypertension is a severe multietiological disease largely resistant to treatment. Biopsy is often necessary to establish differential diagnosis. In pulmonary hypertension transbronchial pulmonary biopsy is contraindicated and open lung biopsy is the method of choice. In our paper we report about experimental results in animals and first clinical data on a new biopsy technique (percutaneous transluminal biopsy, PTB) providing access to histology in pulmonary hypertension without the need of surgery. In a subset of animals pulmonary hypertension was produced experimentally. In addition, we carried out the PTB in patients with bronchial carcinoma. Our preliminary results demonstrate that sufficient material could be gained for the histologic evaluation of pulmonary parenchyma and in pulmonary vessels. No complications have been noted so far.     

Repair of double-outlet right ventricle

The results of repair of double outlet right ventricle in 26 children are reported. Eighteen of these patients had subaortic ventricular septal defect (VSD), without pulmonary stenosis (PS) in 5 and with PS in 13. Another 8 patients had subpulmonic VSD, without PS in 6 and with PS in 2. All had concordant atrioventricular relationships. There were six early deaths and two late deaths of the 26 patients in our study. Of these, 5 patients had severe associated cardiac abnormalities, and 2 who died early had had an incision in the systemic (right) ventricle.