Complex congenital heart disease after in vitro fertilization.
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Adult↗
Biomedical subjects
C Marcelletti
Publications and source records attributed to C Marcelletti.
Bidirectional cavopulmonary shunts: clinical applications as staged or definitive palliation.
A standard Glenn anastomosis between the superior vena cava and the right pulmonary artery has been the accepted mode of treatment for patients with complex cyanotic congenital heart disease. We report our experience in 18 patients with such disease who underwent a bidirectional cavopulmonary shunt because of increasing cyanosis and growth cessation. All patients were considered less than "ideal" candidates for a Fontan procedure. We divided the patients into two groups: group 1 had azygos continuation and group 2 did not. Fourteen patients required hypothermic cardiopulmonary bypass. Bidirectional pulmonary blood flow was achieved in all patients. Only 1 death occurred (group 2). The improvement in oxygen saturation and overall clinical condition of these patients, together with the low mortality and morbidity, is encouraging. However, long-term follow-up is mandatory for a comprehensive evaluation of this surgical approach as definitive palliation or as a first stage for a Fontan operation.
Arteriovenous Shunt, Surgical↗
Anomalous origin of the right pulmonary artery from the ascending aorta: repair without cardiopulmonary bypass.
Three cases of anomalous aortic origin of the right pulmonary artery are reported. All patients presented with severe cardiac failure, two of them in the neonatal period. Clear visualization of the anomalous origin of the right pulmonary artery was obtained by cross-sectional echocardiography and the diagnosis was confirmed by cardiac catheterization. All patients underwent correction without the aid of cardiopulmonary bypass. The continuity between main and right pulmonary arteries was established by a polytetrafluoroethylene graft. One early death was due to bleeding and, probably, a pulmonary vasoconstriction crisis. In all patients the right ventricular pressure was systemic or suprasystemic before surgery and dropped to near normal in the operating room after correction. Two to five years after surgery the survivors are well. This rare, potentially lethal anomaly is amenable to correction; however, diagnosis should be followed by immediate surgical treatment.
Anastomosis, Surgical↗
Palliation of complex cardiac anomalies with subaortic obstruction: new operative approach.
The modified Fontan operation for complex cardiac anomalies associated with subaortic obstruction entails a high surgical risk. It is likely that ventricular hypertrophy secondary to chronic pressure overload plays a significant role. This problem was approached with a new type of palliative operation comprising both a proximal pulmonary artery to ascending aorta anastomosis and a bidirectional cavopulmonary anastomosis. This operation was performed in six children ranging in age from 26 to 63 months. There was one intraoperative death due to hemorrhage. In one patient, a pulmonary to aorta conduit caused compression of the right coronary artery; the problem was solved by lengthening the conduit with a second period of cardiopulmonary bypass. The five survivors experienced an uneventful postoperative course. Repeat cardiac catheterization in these five patients showed low pressure in the cavopulmonary system (mean 10 mm Hg), absence of a gradient at rest between the systemic ventricle and aorta and fair arterial oxygenation (mean 82%). A technetium-99m perfusion lung scan visualized a slight prevalence of pulmonary blood flow ipsilateral to the shunt in three cases, whereas in one case preferential flow to the right lung was associated with a narrowing at the site of the cavopulmonary anastomosis. Mild hypoperfusion of the anterior pulmonary segments was observed in two cases. Both pressure and volume overload are abolished with this procedure and a satisfactory oxygenation is provided. Low venous pressure in the coronary, hepatic and renal areas as well as the short bypass time may explain the smoothness of the postoperative course in our patients.(ABSTRACT TRUNCATED AT 250 WORDS)
Anastomosis, Surgical↗
Discrete subaortic stenosis. Surgery in children based on two-dimensional and Doppler echocardiography.
Twenty pediatric patients underwent surgical resection of a "discrete" subaortic membrane. The diagnosis and the surgical indication were based on two-dimensional and Doppler echocardiography without cardiac catheterization and angiography. In all patients the echocardiographic diagnosis was confirmed at surgery in terms of presence, dimension and location of the membrane and in ten patients in terms of pressure gradients. Two-dimensional and Doppler echocardiography has proved to be a very reliable tool for the diagnosis and surgical indication in pediatric patients with a DSAS. Our criteria for the selection of surgical patients are the following: (1) isolated form of discrete subaortic stenosis with a short base of attachment to the ventricular septum; (2) pressure gradients higher than 25 mm Hg; (3) presence of significant aortic insufficiency. All of this information can be consistently obtained with two-dimensional and Doppler echocardiography.
Aortic Valve Stenosis↗
Congenital mitral valve anomalies in transposition of the great arteries.
Seventy-nine specimens with transposition of the great arteries were reviewed. Among them were 45 with ventricular septal defect, including 6 with so-called "posterior aorta transposition". The morphology of the mitral valve was carefully studied. Mitral valve anomalies were found in 24 cases (30%). The anomalies involving the mitral valve were classified into 4 groups according to the different components of the valve: A) specimens with anomalies of the leaflets--2 cases with mitral cleft; B) those with anomalies of the commissures--1 case with a mitral gap; C) those with abnormalities of the chordae tendinae--2 cases of malattachment of the chordae tendinae to the ventricular septum and 2 with bridging chordae between the papillary muscles; and D) those with abnormalities of the papillary muscles--5 cases with a parachute valve, 10 with a "form fruste" of the parachute mitral valve (hypoplasia of one papillary muscle and short chordae tendinae) and 2 with abnormally placed papillary muscles, which reached the pulmonary annulus. In 14 cases, abnormalities of the tricuspid valve were also encountered, including 8 cases with a tricuspid gap and 6 with a malattachment of tricuspid chordae on the border of a ventricular septal defect. It can be concluded that mitral valve anomalies are not uncommon in transposition of the great arteries. This pathology must be carefully evaluated at the time of surgery, particularly in those patients for whom an anatomic correction is considered, for in those cases the left ventricle will be supplying the systemic circulation and therefore, a completely normal mitral valve will be required.(ABSTRACT TRUNCATED AT 250 WORDS)
Abnormalities, Multiple↗
MEDISYSTEM (R)--a modular computer architecture for integrated management of clinical information and images. Evaluation after one year of use in hospital departments.
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Database Management Systems↗
[Heart transplantation in children: the point of view of the cardiologist].
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Adolescent↗
[Rehabilitation programs for children after congenital heart disease surgery].
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Cardiac Surgical Procedures↗
Tricuspid valve avulsion or excision for right ventricular decompression in pulmonary atresia with intact ventricular septum.
Retrograde right ventricular decompression through the tricuspid valve, by damaging or excising the valve, was attempted in five patients with pulmonary atresia, intact ventricular septum, and severely hypoplastic right ventricle. Two patients were neonates in critical condition, one of whom had received previous palliation with a shunt; the other neonate had received pulmonary valvotomy plus shunt followed by repeat valvotomy plus shunt ligation. Three patients were infants or children who had undergone placement of isolated neonatal shunts. One procedure was performed during cardiac catheterization. Three patients survived. In one, tricuspid avulsion and a Rashkind septostomy were done by percutaneous methods; in two patients, tricuspid excision plus bidirectional cavopulmonary anastomosis was done by open operation. At restudy, all showed subsystemic pressure in the right ventricle and diminution or disappearance of sinusoids. One also showed improvement of left ventricular function. Incorporation of a small right ventricle in the right atrium at the time of the two orthoterminal palliations seemed, at least, innocuous. Right ventricular decompression by tricuspid avulsion or excision could be suggested for all patients with pulmonary atresia, intact ventricular septum, hypersystemic hypoplastic right ventricle, and major sinusoids without right ventricular dependent coronary circulation in whom the outlet portion of the right ventricle is not functional.
Child, Preschool↗
[Seroma: a complication of systemic-pulmonary shunt with a tubular prosthesis].
The accumulation of clear fluid leaking from a tubular prosthesis and confined within a fibrous pseudomembrane has been defined as a "seroma". Both the literature and our experience on this complication have been reviewed. Fifty-three cases of seroma have been reported after implantation of a tubular prosthesis. In our experience, from June 1982 to September 1988 we observed 11 cases (11/108 = 10.1%) of seroma complicating a modified Blalock-Taussig procedure. The mean age of these patients was 2 years 2 months. The diameter of the implanted tubular prostheses was always 5 mm. Among these cases, we report the details of a child, 1 year 10 months old, in whom the seroma significantly complicated the surgical history. According to the data derived from the literature and from our experience, we derived the following risk factors for the occurrence of seroma: age of the patient, size of the prosthesis, surgical manipulations, arterial-venous gradient, transprosthetic flow.
Anastomosis, Surgical↗
[Pulmonary atresia with an intact septum: indications for the neonatal surgical treatment guided by 2-dimensional and Doppler echocardiography].
Twelve neonates with pulmonary atresia and intact ventricular septum underwent surgical treatment based on two-dimensional and Doppler echocardiography. Ten patients with adequate morphology of the right ventricular outflow tract portion shown by means of two-dimensional echocardiography underwent pulmonary valvulotomy and systemic-to-pulmonary shunt. Two neonates with atresia of the right ventricular infundibulum on two-dimensional echocardiography underwent systemic-to-pulmonary artery shunt without valvulotomy. In all patients the qualitative and quantitative echocardiographic diagnosis was confirmed at surgery and/or with the subsequent angiocardiographic study. Two-dimensional and Doppler echocardiography is a precise diagnostic method for planning surgical treatment in neonates with pulmonary atresia and intact ventricular septum.
Echocardiography↗
Long-term results after extracardiac valved conduits implanted for complex congenital heart disease.
Between August 1982 and December 1986, 56 patients survived implantation of an extracardiac valved conduit for complex congenital heart disease. The mean age at operation was 4.2 years (16 days to 24 yrs) and the mean weight was 15.9 kg (2.4 to 93.0 kg). The diagnosis was pulmonary atresia (PA) with ventricular septal defect (VSD) in 13 patients, tetralogy of Fallot in 11, transposition of the great arteries (TGA) with VSD in 8, truncus arteriosus, in 7, complex left ventricular outflow tract obstruction (LVOTO) in 6, complex left atrioventricular valve obstruction in 4, double outlet right ventricle with VSD and subaortic obstruction in 3, univentricular heart with pulmonary stenosis in 2, TGA with LVOTO in 1, and PA with intact ventricular septum in 1. In 35 patients, a preclotted conventional Dacron conduit (CDC) with bioprosthetic valve was used, in 19 patients a collagen-sealed Tascon valved conduit (TC) was implanted, and in 1 patient an aortic homograft was used. In a mean follow-up of 32.5 months (9 to 64 mo), there were two deaths (2/56, 3.6%) that were not related to the conduit. All survivors have been evaluated by two-dimensional and Doppler echocardiography, and 29/56 (51.8%) underwent cardiac catheterization. Nine patients (9/56, 16.1%) underwent successful valved conduit replacement, in seven cases with a nonvalved conduit. There was a significant difference (P = .011) with regard to the incidence of conduit replacement between the group with CDC (2/36, 5.5%) and the group with TC (7/19, 36.8%). Five patients underwent percutaneous transluminal balloon dilatation of the prosthetic conduit, with adequate relief of the gradient in four patients.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
Atrioventricular septal defects with severe left ventricular hypoplasia. Staged palliation.
The natural and surgical history of patients with atrioventricular septal defect, severe left ventricular hypoplasia and right ventricular dominance is poor. We report a successful case of staged surgical palliation based on regulation of the pulmonary blood flow and creation of an unobstructed systemic outlet.
Female↗
Surgical bypass of the systemic atrioventricular valve in children by means of a valved conduit.
The surgical approach to relief of mitral stenosis in children is still a controversial problem. We describe our experience with four severely symptomatic children in whom a valved conduit from the left atrium to the left ventricle was successfully used to bypass a hypoplastic systemic atrioventricular valve. A left atrial-left ventricular extracardiac conduit was implanted in these patients with a hypoplastic mitral anulus and an adequate left ventricular chamber. There were no early or late deaths. Postoperative cardiac catheterization performed in all patients 1 month after the operation showed reduced size of the left atrium, a reduction of the left atrial-left ventricular gradient from a mean of 14 mm Hg to a mean of 5 mm Hg, and an increase of the left atrial outlet from a mean diameter of 10.7 mm to 28.7 mm (including the diameter of the native mitral valve plus the internal diameter of the valved conduit). The application of this unconventional operation in children with congenital or acquired stenosis of the systemic atrioventricular valve should be considered when the mitral valve obstruction cannot be relieved by conventional valve repair or replacement. Furthermore, the left atrial-left ventricular conduit does not preclude future alternative surgical options.
Aorta↗
Palliative Mustard operation: an expanded horizon.
From March 1982 to December 1983, five patients with a mean age 7 years (4 months-16 years) underwent a palliative Mustard operation for complex cardiac anomalies. The diagnoses were: univentricular heart with pulmonary vascular obstructive disease (PVOD) (2 cases); criss-cross heart with intact atrial septum, ventricular septal defect (VSD), transposition of the great arteries (TGA) and moderate pulmonary stenosis; isolated atrioventricular discordance with VSD; TGA, diminutive right ventricle and multiple VSDs. Cardiac catheterization showed unfavourable direction of flow: the oxygen saturation in the aorta was lower than in pulmonary artery, with a mean difference of 13.5% (range 6% to 30%). In no case was anatomical or physiological repair considered advisable because of the unfavourable intracardiac anatomy or the presence of PVOD. In two patients the atrial rerouting was accompanied by pulmonary artery banding. There were no hospital or late deaths in a mean follow-up of 36 months (range 29 to 50 months). The mean post-operative arterial oxygen saturation was 95.4% (range 92% to 99%).
Adolescent↗
Indication for systemic-pulmonary artery shunts guided by two-dimensional and Doppler echocardiography: criteria for patient selection.
From June, 1983, to February, 1987, 91 patients with pulmonary outflow tract obstruction underwent a systemic-to-pulmonary artery shunt without an invasive study. The surgical indication was based on two-dimensional (2-D) and Doppler echocardiography only. In 82 patients (90.1%), 2-D and Doppler echocardiography allowed correct assessment of the intracardiac anatomy and of the morphology of the pulmonary arteries, pulmonary veins, and aortic arch. The aortic arch anatomy was misdiagnosed in 6 patients (6.6%) who underwent a modified instead of a classic Blalock-Taussig shunt. In 3 patients (3.3%), the indication for the systemic-pulmonary artery shunt was inappropriate; in 1, operative death resulted from the incomplete diagnosis. After the preselection of patients based on knowledge of the anatomical patterns, a systemic-pulmonary artery shunt may be performed without cardiac catheterization if these rules for definitive patient selection are followed: (1) accurate clinical assessment to identify the patients with restricted pulmonary blood flow; (2) precise intracardiac diagnosis by 2-D and Doppler echocardiography; and (3) definitive 2-D echocardiographic visualization of the pulmonary venous connection and the confluence of the pulmonary arteries.
Anastomosis, Surgical↗
Palliation of cardiac malformations associated with right isomerism (asplenia syndrome) in infancy.
Right isomerism is characterized by the combination of an obstructed pulmonary outflow tract and a total anomalous pulmonary venous connection (TAPVC), which is obstructed in nearly half of the patients. Fourteen patients less than 2 years of age with right isomerism have been seen in our unit. Thirteen of them underwent palliation consisting of a systemic-pulmonary shunt (10 patients) or a combination of shunt and TAPVC repair (3). There were 7 hospital deaths (54%). The presence of obstructed pulmonary venous drainage was the major risk factor in the surgical treatment of these complex cardiac anomalies (p less than .02). Guidelines for the palliative management of right isomerism are suggested.
Echocardiography↗