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.     

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.     

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.     

Left ventriculotomy for closure of muscular ventricular septal defects. Treatment of choice

Closure of muscular ventricular septal defects (VSDs) through the right atriotomy or right ventriculotomy may be difficult. These VSDs are often located behind the hypertrophied trabeculae carnae or papillary muscle. Residual or recurrent VSD may result from the difficult approach. Between March 1971 and December 1975, we have used the left ventriculotomy near the apex for closure of muscular VSDs in ten children. The patients' ages ranged from five months to eight years and three months. The diagnosis was established by cardiac catheterisation and left ventricular angiocardiogram in all patients. Six patients had multiple VSDs; in four patients VSD in the muscular septum was present (three apical, one midseptal). Operations were performed on cardiopulmonary bypass with moderate hypothermia and intermittent anoxic arrest. VSDs in the membranous septum were closed through the right atrium. Muscular VSDs were approached through a small vertical incision in the left ventricle near the apex. The postoperative course was uneventful in eight patients. Two patients, aged 16 months and eight years, died; histology showed grade IV pulmonary vascular disease in both. All survivors are well four months to five years after the operation, without clinical evidence of residual or recurrent VSD.     

Mitral valve anomalies obstructing left ventricular outflow

This paper reports on two cases of more uncommon types of subaortic stenosis. A 2-year-old boy was found with accessory mitral valve leaflet (AMVL) attaching to the anterior leaflet, ballooning into the subaortic ventricular septum associated with a discrete subaortic membrane. The obstruction was successfully relieved by removal of the AMVL and resection of the membrane. A 19-day-old newborn with accessory tissue on the mitral valve (AMVT) causing subaortic stenosis, subaortic ventricular septal defect (VSD) and patent ductus arteriosus was operated on successfully. Accessory tissue excision through the VSD, VSD patch closure and ductus ligation were performed.     

Persistence of fetal circulation syndrome: an echocardiographic study

Serial echocardiograms were performed on 17 infants with persistence of fetal circulation syndrome to measure right ventricular systolic time intervals from pulmonic valve echograms and left ventricular systolic time intervals from aortic valve echograms. Right ventricular pre-ejection period/right ventricular ejection time ratio was prolonged in PFCS when compared to that in normal newborn infants, and diminished with clinical improvement. Left ventricular pre-ejection period/left ventricular ejection time ratio was prolonged in infants with PFCS. Echographic RPEP/RVET was consistent with the elevated pulmonary artery pressure and pulmonary vascular resistance of PFCS; elevated LPEP/LVET suggested left ventricular dysfunction.     

Destructive gouty arthritis of the hip

We present a case of a 19-year old female with systemic pulmonary artery (PA) pressure due to a congenital ventricular septal defect (VSD) and atrial septal defect (ASD). She was pink at rest and cyanotic on exercise. Lung biopsy revealed grade IV pulmonary vascular changes. As a preliminary step PA was banded to increase right-to-left shunt and decrease aortic (Ao) saturation with consequent decrease in PA saturation. After one year, when she was no longer cyanotic, even on exercise, lung biopsy revealed total regression of pulmonary vascular changes. As a definitive procedure VSD and ASD were closed and PA was debanded. Cardiac catheterization one week postoperatively showed PA pressure to be 50% of systemic pressure. We postulate that reversal of pulmonary vascular changes were due to lowered PA saturation. We further believe that lower PA pressure could have contributed to this regression of pulmonary vascular changes. We performed the same procedure in six more patients with similar positive clinical response. This new concept brings renewed hope to many children who otherwise are candidates for heart lung transplantation.     

In-vitro isolation and antifungal susceptibility of amphotericin B-resistant mutants of Aspergillus fumigatus

BACKGROUND AND AIMS OF THE STUDY: Aortic valve disease in the pediatric population poses special problems to surgeons and cardiologists. The pulmonary autograft has proven to be a good alternative for aortic valve replacement and left ventricular outflow tract (LVOT) reconstruction in this special group. METHODS: Forty-one children (mean age 10.0 +/- 4.8 (SD) years; range: 35 days to 18.8 years) underwent aortic root replacement with a pulmonary autograft between February 1994 and April 1998. Twenty-one patients (51%) had previous cardiac surgery; seven (17%) had balloon valvulotomy. Aortic root replacement was combined with other techniques for various disorders, including tunnel LVOT obstruction, ventricular septal defect (VSD)-aortic insufficiency complex, neoaortic insufficiency following arterial switch procedure, and subvalvular stenosis following correction of type B interruption of the aortic arch (IAA) with VSD (IAA-B/VSD). RESULTS: The mean follow up was 1.7 +/- 1.0 years (range 44 days to 4.1 years). Total follow up time was 67.8 patient-years. Two patients, both after repair of interrupted aortic arch, died intraoperatively (4.9%). There was no late mortality. Two patients were reoperated on (5.1%), one for autograft insufficiency due to cuspal perforation and one for right ventricular outflow tract stenosis at the distal anastomosis. Thirty-eight patients (97%) are currently in NYHA class I; one child with a preoperatively poor left ventricular function did not improve and is in class II. At the latest echocardiographic follow up, neoaortic regurgitation was absent in 19% of patients, trivial in 69% and mild in 11%. Homograft insufficiency was absent in 64%, trivial in 31% and mild in 6%. All mean gradients for both autograft and homograft were < 15 mmHg. CONCLUSIONS: The Ross operation can be performed with good results in infants and children with different forms of LVOT obstruction and aortic insufficiency, though aortic stenosis following IAA-B/VSD repair poses a surgically difficult problem.     

Perioperative management of pulmonary hypertension after heart transplantation in childhood

BACKGROUND: Pulmonary hypertension is responsible for a substantial part of perioperative and postoperative mortality and morbidity after cardiac transplantation. Treatment of right ventricular failure after increased pulmonary vascular resistance is difficult especially in infants and children. Therefore we started a preventive therapy of pulmonary hypertension after cardiac transplantation to avoid right ventricular failure and compared the results with a group of patients with conventional therapy. METHODS: Group 1 (n = 13), with transplantation from 1988 to 1991, was treated with vasodilators when symptoms of right ventricular failure developed. Group 2 (n = 19) had preventive treatment with prostaglandin E1 (PGE1), the phosphodiesterase-III inhibitor enoximone, and alkalinazation starting during weaning from cardiopulmonary bypass. RESULTS: Six patients in group 1 died; four of them as the result of right ventricular failure in the immediate postoperative course despite aggressive treatment. In group 2 there were three deaths as the results of rejection (2) and infection (1). None of these patients developed right ventricular failure (p = 0.02). Cold ischemic time, extracorporeal circulation time, and waiting time before transplantation were significantly longer in group 2. Side effects of this preventive therapy were not observed. CONCLUSIONS: We conclude that prophylactic therapy of pulmonary hypertension with vasodilators in infants and children after heart transplantation is safe and effective in preventing right ventricular failure in the postoperative course.     

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.     

Cases of multiple ventricular septal defects

We had 4 cases with multiple ventricular septal defects (VSDs) in complexed congenital heart disease. One of four had two separate VSDs detected by two dimensional echocardiography before operation. Second of four had additional infundibular muscular VSD which was detected by echocardiography in the intensive care unit (ICU) after patch closure of a perimembranous VSD. The third case had two additional VSDs of inlet muscular and subaortic septum detected by transesophageal and direct echocardiography during reoperation, beside a subpulmonary VSD which was originally diagnosed before Jatene operation for double outlet right ventricle. The fourth case had multiple trabecular muscular VSDs diagnosed by postoperative angiography soon after Rastelli operation. Since these additional multiple VSDs compromise the postoperative hemodynamics if those are unrecognized, it is indispensable to detect all VSDs before operation, using transthoracic and transesophageal echocardiography.     

Conversion of complex neonatal Ebstein's anomaly into functional tricuspid or pulmonary atresia

BACKGROUND: Ebstein's anomaly, due to failure of delamination of one or more leaflets of the tricuspid valve (TV), is associated with varying degrees of tricuspid regurgitation (TR) and dysplasia of the right ventricle (RV). Although refinement of tricuspid valvuloplasty and plication techniques have opened the way to a satisfactory outlook for the majority of older children and adults, Ebstein's anomaly presenting at neonatal age, secondary to ineffective forward flow into the pulmonary and systemic circulation, has a reported mortality rate of as high as 75%. In order to improve the dismal outcome in neonatal Ebstein's anomaly, we have strived for early univentricular palliation. PATIENTS AND METHODS: Univentricular repair was performed in five neonates (median age 5 days; range 2-14 days) with Ebstein's anomaly, ductal dependent pulmonary blood flow, severe TR, absence of forward flow across the pulmonary valve, and small left ventricular (LV) area due to right-to-left bowing of the ventricular septum and ineffective LV loading (median indexed LV area 10.5 cm2/m2). In addition, two neonates had moderate pulmonary regurgitation (PR), one with additional pulmonary stenosis. In all patients, the indexed area of the combined right atrium and atrialized RV was greater than that of the combined functional RV, left atrium, and left ventricle (median 22.0 and 20.8 cm2/m2, respectively). The median preoperative systemic oxygen tension was 35 mmHg and the median pH 7.28. Repair consisted of TV closure with a pericardial patch (with the coronary sinus draining into the RV) (n = 3) or, in the presence of PR, resection of the dysplastic TV and division and oversewing of the main pulmonary artery (n = 2), as well as excision of the atrial septum, resection of redundant right atrial wall, and construction of an aortopulmonary shunt (n = 5). RESULTS: The median indexed LV area increased from 10.5 to 18.8 cm2/m2 as a result of more effective loading of the left ventricle. There was no intraoperative or late mortality. The patients were extubated at a median of 7 days postoperatively. At discharge, the median systemic oxygen tension was 46 mmHg. In all five patients, at 6, 7, 10, 12 and 16 weeks of age, a bidirectional cavopulmonary anastomosis has been constructed. CONCLUSIONS: In neonates with Ebstein's anomaly and ductal dependent pulmonary blood flow, rational palliation consists of the surgical creation of tricuspid atresia or, in the additional presence of PR or pulmonary stenosis, the creation of pulmonary atresia. These procedures may result in effective LV decompression and more effective volume loading of the left ventricle with increase of systemic output and improved clinical outcome.     

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.     

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.     

Suspension of straddling tricuspid valve chordae into the appropriate ventricle

In 2 children with an inlet ventricular septal defect and straddling chordae tendineae of the septal leaflet of the tricuspid valve to the posteromedial papillary muscle of the mitral valve and to an accessory papillary muscle in the left ventricle, the straddling chordae were excised with a wedge of posteromedial papillary muscle and with the top segment of the accessory papillary muscle, respectively. After patch closure of the ventricular septal defect, the papillary muscle segment with its group of chordae was anchored to the right ventricular septum with resulting competence of the tricuspid valve. In contrast to the traditional repair technique, the reported modification is applicable when the straddling chordae insert into a papillary muscle of the mitral valve. In addition, various disadvantages related to the construction of a complex baffle in the inappropriate ventricle are avoided.     

Inhaled nitric oxide, right ventricular efficiency, and pulmonary vascular mechanics: selective vasodilation of small pulmonary vessels during hypoxic pulmonary vasoconstriction

OBJECTIVE: In the setting of acute pulmonary artery hypertension, techniques to reduce right ventricular energy requirements may ameliorate cardiac failure and reduce morbidity and mortality. Inhaled nitric oxide, a selective pulmonary vasodilator, may be effective in the treatment of pulmonary artery hypertension, but its effects on cardiopulmonary interactions are poorly understood. METHODS: We therefore developed a model of hypoxic pulmonary vasoconstriction that mimics the clinical syndrome of acute pulmonary hypertension. Inhaled nitric oxide was administered in concentrations of 20, 40, and 80 ppm. RESULTS: During hypoxic pulmonary vasoconstriction, the administration of nitric oxide resulted in a significant improvement in pulmonary vascular mechanics and a reduction in right ventricular afterload. These improvements were a result of selective vasodilation of small pulmonary vessels and more efficient blood flow through the pulmonary vascular bed (improved transpulmonary vascular efficiency). The right ventricular total power output diminished during the inhalation of nitric oxide, indicating a reduction in right ventricular energy requirements. The net result of nitric oxide administration was an increase in right ventricular efficiency. CONCLUSION: These data suggest that nitric oxide may be beneficial to the failing right ventricle by improving pulmonary vascular mechanics and right ventricular efficiency.     

Surgical treatment of ventricular septal defect and ruptured sinus of Valsalva associated with infective endocarditis

Two patients with ventricular septal defect of Kirklin type I and ruptured right coronary sinus of Valsalva associated with infective endocarditis were operated on. Both had bacillus vegetation clinging to the aortic and pulmonary valves and the right ventricular intimal wall around the septal defect. Aortic and pulmonary regurgitation were also found. The surgical approach included vertical incision of the right ventricular outflow tract and pulmonary trunk and transverse aortotomy. The right coronary sinus of Valsalva showed distinct aneurysmal change in one patient. The aortic valve and infected Valsalva sinus were excised in both cases, and the pulmonary valve and right ventricular wall where infection extended thoroughly débrided. The resulting defect, including the ventricular septal defect and excised right Valsalva sinus and aortic annulus, was closed with one patch, and the prosthetic valve inserted in the position of the original aortic valve using this patch as part of the annulus. Both patients had a good postoperative course and are doing well, although slight pulmonary regurgitation persists.     

Homografts in congenital heart disease: current applications and future directions

The first clinical use of homograft tissue in cardiovascular surgery was in 1948, when Gross used cadaveric arterial grafts to construct systemic to pulmonary artery shunts in patients with tetralogy of Fallot, and to repair coarctation of the aorta. Eighteen years later, a valved homograft was used for the first time in the treatment of congenital heart disease for reconstruction of the right ventricular outflow tract in a child with pulmonary atresia. Since these pioneering advances, valve and vascular homografts have become central to the management of congenital anomalies of the heart and great vessels. The primary use for homografts in congenital heart surgery today is establishment of a valved connection between the right ventricle and pulmonary arteries in children with tetralogy of Fallot with pulmonary atresia or other complicating factors, truncus arteriosus, transposition complexes, and double-outlet right ventricle, and in patients undergoing the Ross procedure. Homograft reconstruction of the left ventricular outflow tract has also been performed for many years in children with aortic insufficiency or recurrent aortic stenosis, but early homograft degeneration in young children has been a significant problem. Many surgeons are turning away from homografts in the systemic circulation in favor of the pulmonary autograft. Homograft is also widely used as a vascular patch material. In the present report, we discuss the various uses of homografts in congenital heart surgery, the benefits and drawbacks of homografts in young patients, and some of the future possibilities for homograft development and application in patients with congenital heart disease.