Effect of coexistent coarctation of pulmonary trunk in natural history of complete absence of pulmonary valve with ventricular septal defect.

A case of complete absence of the pulmonary valve in association with a ventricular septal defect and a pulmonary artery coarctation is described. Despite these defects the patient had minimal symptoms, probably because the pulmonary artery coarctation acted as a natural banding and limited the pulmonary regurgitant flow and reduced the left-to-right shunt across the ventricular septal defect. The patient was operated on at the age of 40 and the ventricular septal defect was closed but the pulmonary artery coarctation was left alone. In view of the long-term survival in this case, it is suggested that pulmonary artery banding would reduce the symptoms and increase the life expectancy of patients with congenital absence of the pulmonary valve and a ventricular septal defect.     

Obstructions to pulmonary ejection and restrictive interventricular septal defects

The association of a restrictive ventricular septal defect with a pulmonary tract obstruction is rare and may cause diagnostic and therapeutic problems. The authors report their experience of such an association in 10 children. 3 had a tetralogy of Fallot (1 death), 4 a double-outlet right ventricle (2 deaths), and 3 a coarctation of the aorta with ventricular septal defect and pulmonary artery banding (no death). Prognosis depends on the type of malformation. It has improved with years because of sooner recognition of these malformations and better surgical technics and post-operative care.     

Surgical management of coarctation of aorta with ventricular septal defect. Multivariate analysis.

Optimal management of coarctation with ventricular septal defect is difficult. Should one treat the coarctation, the ventricular septal defect, or both? This dilemma was investigated by reviewing 39 successive patients, aged less than 4 months, undergoing coarctation repair without pulmonary artery banding. Twelve hospital deaths occurred. Ventricular septal defect size was graded "blind" according to preoperative angiocardiographic and haemodynamic findings. Of 14 patients classified as having a large ventricular septal defect, necropsy and operative findings in eight showed defects 7 to 12 mm in diameter. Factors associated univariately with significantly increased mortality were young age, raised atrial and ventricular end-diastolic pressures, low weight, high admission blood urea, preoperative ventilation, and a large ventricular septal defect. All but the last two were also closely associated with each other. A jack-knifed discriminant function based upon ventricular septal defect size, blood urea, pulmonary venous oxygen content, and inferior caval oxygen saturation correctly predicted outcome in 78.9% of patients. Combinations of these four giving a probability greater than 0.9 of survival were rare with blood ureas above 8 mmol/litre. Raised blood urea was associated with low descending aortic pressure and subsequent dialysis. Only patients with a large ventricular septal defect stand to benefit from pulmonary artery banding at initial operation. Preoperative treatment to increase renal blood flow (prostaglandins, dopamine) may improve overall survival.     

Successful surgical repair of aortic atresia associated with normal left ventricle

Surgical repair of aortic atresia with a large ventricular septal defect and a normally developed left ventricle was accomplished in a 14 month old infant. Palliative surgery at age 23 days consisted of bilateral banding of pulmonary artery branches and replacement of the ductus arteriosus with a Goretex conduit. Corrective surgery was accomplished by closure of the ventricular septal defect, insertion of a valved conduit between the apex of the left ventricle and the subdiaphragmatic aorta, removal of the pulmonary artery bands and division of the pulmonary-aortic conduit. The patient has a mild coarctation of the aorta and remains asymptomatic at 2 years of age.     

Surgical management of the infant with coarctation of the aorta and ventricular septal defect.

Clinical and cardiac catheterization data were collected from 39 infants with coarctation of the aorta and ventricular septal defect, 31 of whom were initially managed only by surgical repair of coarctation. Data were analyzed to determine mortality, morbidity, outcome and factors that might predict survival or the need for septal defect closure. Of the eight patients who did not require surgical treatment before 3 months of age, seven underwent coarctation repair alone at a mean age of 2.3 years. Of the 23 infants managed with coarctation repair alone, before age 3 months, 9 needed no additional surgical treatment and 6 required early and 8 required late repair of the ventricular septal defect. Seven infants underwent coarctation repair and simultaneous pulmonary artery banding and one eventually required debanding after spontaneous closure of the septal defect. The overall mortality rate in this series was 10.3% (mean follow-up time 5.7 years). Of 39 infants, 16 (41%) never required a second operation for ventricular septal defect closure. For patients who had only coarctation or coarctation repair with pulmonary artery banding at less than 3 months of age, ventricular septal defect size was categorized as small (less than 0.5 cm/m2), moderate (less than 1 cm/m2) or large (greater than 1 cm/m2) on the basis of defect size at operative repair or echocardiographic or angiographic assessment. Defect size did not necessarily correlate with the need for operative repair.(ABSTRACT TRUNCATED AT 250 WORDS)     

Morphological characterisation of ventricular septal defects associated with coarctation of aorta by cross-sectional echocardiography.

Cross-sectional echocardiograms of 18 neonates and infants with coarctation of the aorta and ventricular septal defect were retrospectively assessed. With a combination of subcostal and precordial cuts the site and anatomical relations of the defects were determined. In one case there was a muscular trabecular ventricular septal defect. Three had a malalignment defect with associated left ventricular outflow tract obstruction. In two there was a doubly committed subarterial defect with associated malalignment of the point of continuity between the aortic and pulmonary valves and the crest of the trabecular septum. Twelve cases had a perimembranous defect, with varying degrees of extension into the inlet, trabecular, or outlet septum. In this group 10 had associated aortic override, with varying degrees of left ventricular outflow tract narrowing. Abnormal insertion of the tricuspid valve was observed in 10 cases, such that it partly obscured the ventricular septal defect. Thus in most cases of coarctation with ventricular septal defect, the morphology of the ventricular septal defect and ventricular outflow tracts is such that left ventricular ejection is directed towards the pulmonary artery rather than the aorta.     

A new strategy for the surgical treatment of aortic coarctation associated with ventricular septal defect in infants using an absorbable pulmonary artery band.

OBJECTIVES: We propose a new strategy using coarctation repair together with a polidioxanone absorbable pulmonary artery banding to limit operative risk and to spare infants with aortic coarctation subsequent operations. BACKGROUND: The alternative for the surgical management of aortic coarctation associated with ventricular septal defect (VSD) is single-stage repair versus coarctation repair with or without banding of the pulmonary artery. METHODS: Eleven infants (mean weight 2,560 +/- 1,750 g, range 1,320 to 3,800 g) underwent a coarctation repair with a polydioxanone banding. Seven had a trabecular and four a perimembranous VSD. The mean size of the VSD was 5 +/- 0.7 mm (range 4 to 7 mm). The systolic pulmonary pressure was >80% of the aortic pressure in all. The pulmonary band was tightened until the systolic pulmonary pressure fell below 50% of the aortic pressure. RESULTS: There were no hospital deaths. The reabsorption of the banding was complete after 5.7 months in all patients (3 to 6.5 months). The VSD closed completely in four infants and partially in six, in whom the pulmonary artery pressure was normal without evidence for significant left-to-right shunt. One patient with a large trabecular VSD underwent surgical closure of his defect after four months. Finally, a subsequent open-heart surgery could be avoided in 91% (10/11) of patients. CONCLUSIONS: Provided the VSD belongs to types prone to close spontaneously, this policy may reduce the number of surgical procedures per infant as well as in-hospital mortality and morbidity rates. It should be proposed as an alternative to more complex procedures.     

Second heart sound after pulmonary arterial banding operation.

After a pulmonary arterial banding procedure the phonocardiograms of 38 patients were correlated with haemodynamic and angiographic findings. Twenty-four patients had uncomplicated ventricular septal defect, 2 had single ventricle, 5 had transposition of the great arteries, 5 had atrioventricular canal defects, and 2 had coarctation of the aorta and ventricular septal defect. P2 was separated from A2 by less than 40 ms in 10 of the 11 patients with high pulmonary vascular resistance. Of 27 patients with nearly normal pulmonary vascular resistances and distal pulmonary artery pressures less than 50/20 mmHg (6-7/2-7 kPa), 24 had A2-P2 intervals of over 40 ms. A narrow A2-P2 interval with a satisfactory band was found in 2 patients with large right-to-left shunts. A2-P2 interval did not change appreciably with age, and this measurement is a useful guide as to the effectiveness of pulmonary artery banding by one year after operation. If this interval is less than 40 ms, repeat catheterization should be carried out as such patients may have persisting pulmonary hypertension and an increased pulmonary vascular resistance.     

Surgical correction of ventricular septal defects treated by banding the pulmonary artery. Apropos of 38 cases]

The authors report their experience with two-stage surgical treatment of severe ventricular septal defects in babies: initial banding of the pulmonary artery, followed by closure of the ventricular septal defect and removal of the band by an open-heart technique. Out of 162 operations to band the pulmonary artery, 90 were for cases of ventricular septal defect. The mortality was low (9.1%) when there was a ventricular septal defect with or without a patent ductus. It is considerably higher (31.4%) when there is a coincident coarctation of the aortic isthmus. So far 38 children have had the second operation, with 2 deaths. The authors have studied the surgical problems and the long-term results (6 months to 3 years follow-up) in the first 29 patients undergoing surgery. Although the results from this two-stage operation are good, when the authors take into account their current experience with one-stage closure of ventricular septal defects in the babies, they feel that this latter technique is to be preferred. Reservations must, however, be expressed when the child is very young (Weighing 3.5 kg or less), when there are multiple defects between the ventricles, and when the defect is severe, with a combination of VSD, patent ductus and coarctation of the aorta, and sometimes when the opertion must be carried out as an emergency.     

Second heart sound after pulmonary arterial banding operation.

After a pulmonary arterial banding procedure the phonocardiograms of 38 patients were correlated with haemodynamic and angiographic findings. Twenty-four patients had uncomplicated ventricular septal defect, 2 had single ventricle, 5 had transposition of the great arteries, 5 had atrioventricular canal defects, and 2 had coarctation of the aorta and ventricular septal defect. P2 was separated from A2 by less than 40 ms in 10 of the 11 patients with high pulmonary vascular resistance. Of 27 patients with nearly normal pulmonary vascular resistances and distal pulmonary artery pressures less than 50/20 mmHg (6-7/2-7 kPa), 24 had A2-P2 intervals of over 40 ms. A narrow A2-P2 interval with a satisfactory band was found in 2 patients with large right-to-left shunts. A2-P2 interval did not change appreciably with age, and this measurement is a useful guide as to the effectiveness of pulmonary artery banding by one year after operation. If this interval is less than 40 ms, repeat catheterization should be carried out as such patients may have persisting pulmonary hypertension and an increased pulmonary vascular resistance.     

Infective endocarditis of pulmonary valve. Considerations apropos of a case associated with interventricular communication and aortic insufficiency

We report the case of a 23 year old patient in whom heart disease was detected in childhood. He began to have suddenly fever and symptoms of right side heart failure. There were signs of aortic regurgitation, a systodiastolic murmur in the left parasternal region and a diastolic murmur in the left 4th intercostal space. The bidimensional and Doppler Echocardiogram showed a saccular dilatation in the right ventricular outflow tract. He was submitted to catheterisation which confirmed aortic regurgitation and detected ventricular septal defect and pulmonary hypertension. During surgery, a subaortic ventricular septal defect was seen as well as a dilatation of the right coronary aortic cusp which prolapsed through the defect to the right ventricular outflow tract. Vegetations were seen in the pulmonary valve. The aortic valve and a pulmonary cusp were excised and a Bj?rk Shiley aortic prosthetic valve was inserted. The septal defect was closed. The patient is still alive, symptomless, with 40 months of follow up. We discuss some problems related to this case, particularly the surgical indication and the rarity of the morphology discovered.     

Coarctation of the aorta in early infancy. Left ventricular outflow tract obstruction and its clinical implications

In order to clarify the clinical significance of left ventricular outflow tract obstruction, 11 infants with coarctation of the aorta and ventricular septal defect were subjected to cineangiography (or autopsy) analysis. Three infants without left ventricular outflow tract obstruction were salvaged by aortoplasty using a subclavian flap or a Dacron patch with concomitant pulmonary artery banding. The remaining 8 infants died surgically or nonsurgically, and were confirmed retrospectively to have had left ventricular outflow tract obstruction. It seems worthwhile to stress that the presence of an abnormal muscle bundle in the left ventricle can be demonstrated angiographically, especially by the axial technique. From our present study, though the number of cases examined is limited, we have an impression that left ventricular outflow tract obstruction may be a determining factor with regard to the appropriateness of pulmonary artery banding in the two-stage operation for infantile coarctation associated with a large ventricular septal defect.     

Congenital absence of the pulmonary valve

The clinical, radiological, electrocardiographic, and haemodynamic findings in 7 patients with the syndrome of congenital absence of the pulmonary valve with ventricular septal defect are reported. Findings included a pansystolic murmur and a low-pitched delayed diastolic murmur at the left sternal border with no pulmonary valve closure sound, an rsR′ pattern in V1 on the electrocardiogram, and a chest x-ray showing a large heart and gross dilatation of the main, left or right pulmonary arteries but normal or reduced vascularity of the peripheral lung fields. Cardiac catheterization showed a left-to-right shunt at ventricular level in every case, though in one the shunt was bidirectional. Despite right ventricular systolic pressures at least 75 per cent of systemic level in every patient, the pulmonary artery pressure was invariably normal. Pulmonary stenosis was shown to exist at infundibular level, at valve ring level, or at both. Selective right ventricular angiography was diagnostic: the main and right or left pulmonary arteries were aneurysmally dilated and there was an abrupt transition in calibre between these vessels and their immediate branches; enlargement of the right pulmonary artery caused distortion and downward displacement of the left atrium in 4 patients; in 2 there was a flat `diaphragm' in the pulmonary valve position.     

Congenital absence of the pulmonary valve

The clinical, radiological, electrocardiographic, and haemodynamic findings in 7 patients with the syndrome of congenital absence of the pulmonary valve with ventricular septal defect are reported. Findings included a pansystolic murmur and a low-pitched delayed diastolic murmur at the left sternal border with no pulmonary valve closure sound, an rsR' pattern in V1 on the electrocardiogram, and a chest x-ray showing a large heart and gross dilatation of the main, left or right pulmonary arteries but normal or reduced vascularity of the peripheral lung fields. Cardiac catheterization showed a left-to-right shunt at ventricular level in every case, though in one the shunt was bidirectional. Despite right ventricular systolic pressures at least 75 per cent of systemic level in every patient, the pulmonary artery pressure was invariably normal. Pulmonary stenosis was shown to exist at infundibular level, at valve ring level, or at both. Selective right ventricular angiography was diagnostic: the main and right or left pulmonary arteries were aneurysmally dilated and there was an abrupt transition in calibre between these vessels and their immediate branches; enlargement of the right pulmonary artery caused distortion and downward displacement of the left atrium in 4 patients; in 2 there was a flat ;diaphragm' in the pulmonary valve position.The diagnosis was confirmed in 3 patients by necropsy. The pulmonary valve was represented by at most a rudimentary fringe of tissue. Histological examination of the pulmonary arteries in one patient showed a correlation between the dilatation of the artery and the structure of its wall.     

6kg以下小婴儿主动脉缩窄合并室间隔缺损的外科矫治

目的 :探讨新生儿、小婴儿主动脉缩窄 (CoA)合并室间隔缺损 (VSD)的外科治疗方法。方法 :1998年 12月至 2 0 0 3年 7月共收治 9例 6kg以下CoA合并VSD患儿 ,其中新生儿 3例。一期手术 3例 ,分期手术 3例 ,仅做主动脉缩窄成形术 3例 ,其中 1例加做肺动脉环缩术。 6例采用人造血管补片主动脉成形术 ,3例采用左锁骨下动脉翻转主动脉成形术。结果 :全组患儿无死亡 ,随访 8～ 36个月 ,心功能改善明显 ,超声心动图检查提示无主动脉瘤样扩张或主动脉再狭窄。结论 :大多数患儿均可采用体外循环下一期根治CoA和VSD ,但是对于VSD为限制性及不能耐受体外循环的患儿可采用二期手术。一期手术选择左外侧和正中双切口方法安全有效。     

Clinical presentation, natural history, and outcome of patients with the absent pulmonary valve syndrome

So-called 'absent pulmonary valve syndrome' is a rare cardiac malformation, usually associated with tetralogy of Fallot. Congenital absence of the leaflets of the pulmonary valve is less common when the ventricular septum is intact. Characteristic features of the syndrome include dysplasia or absence of the pulmonary valvar leaflets, permitting severe pulmonary regurgitation, and aneurysmal dilation of the pulmonary arteries. The purpose of our study was to review our experience with patients diagnosed as having the absent pulmonary valve syndrome, and to describe their clinical presentation, natural history, and outcome. We reviewed retrospectively data from 18 patients with absent pulmonary valve syndrome, 10 boys and eight girls, treated between March 1983 and May 2003. We identified two groups of patients, one made up of 11 patients with a ventricular septal defect, in whom the morphology of the subpulmonary outflow tract was phenotypic for tetralogy of Fallot, and another group, with seven patients, having an intact ventricular septum. Family history of congenital heart disease was common only in patients with ventricular septal defect, being found in 73%, all of whom were diagnosed during infancy with variable respiratory distress. Diagnosis was delayed in 43% of the patients with an intact ventricular septum. Cardiac surgery was performed in eight patients with ventricular septal defect (73%), compared to only two patients (28%) with an intact ventricular septum. Overall mortality was 28%, with five patients dying. Although our sample was small, two clinical patterns emerged depending on the presence or absence of a ventricular septal defect. Patients with a ventricular septal defect and phenotypic features of tetralogy of Fallot have a strong family history of congenital cardiac disease, develop respiratory symptoms during infancy and exhibit a variable prognosis, despite cardiac surgery. Patients with an intact ventricular septum are usually asymptomatic, present later in life, and show a relatively benign prognosis.     

Coarctation of the aorta in infancy

Twenty-five infants under 1 year of age (mean weight 3.4 kg) underwent repair of coarctation of the aorta between the years 1965 and 1982. Three patients had coarctation only, three had coarctation with patent ductus arteriosus (PDA), and 19 had associated intracardiac anomalies. Eleven patients underwent resection of the aorta and end-to-end anastomosis. Eight had subclavian flap arterioplasty, five had patch graft arterioplasty, and one had subclavian-to-aortic anastomosis. Additional procedures were performed on seven patients: banding of the pulmonary artery on one, repair of total anomalous pulmonary venous drainage on one, mitral valve replacement on one, aortic valvotomy on one, and aortic valvotomy plus pulmonary artery banding on one. Twenty-one survived the operation. All patients who died had associated intracardiac anomalies. The 21 survivors have been followed from 3 months to 13 years, with three late deaths that were associated with intracardiac anomalies. Five of the survivors underwent additional second operations: one had repair of the re-coarctation, one had replacement of the prosthetic mitral valve, one had aortic valvotomy, and two had pulmonary artery debanding and closure of a ventricular septal defect. Two of the 18 surviving patients have mild hypertension associated with a residual gradient. The others are in good health.     

Total anomalous pulmonary venous drainage with ventricular septal defect

Three patients with total anomalous pulmonary venous drainage and ventricular septal defect with left-to-right shunt are presented. The ventricular septal defect was diagnosed with left ventricular angiocardiography in two patients and confirmed on postmortem examination in the third.The complex hemodynamic implications of this combination of lesions are discussed. Pulmonary artery banding is suggested as a possible method of management in early infancy.When total anomalous pulmonary venous drainage is demonstrated in infants initially suspected of having large ventricular septal defects on the basis of auscultation, left ventricular angiocardiography is mandatory. Preoperative diagnosis of a complicating ventricular septal defect is essential to optimal management.     

Isolated pulmonic valve endocarditis in an adult patient with ventricular septal defect and infundibular pulmonary stenosis

Ventricular septal defect associated with infundibular pulmonary stenosis is a relatively uncommon congenital cardiac defect. We report the first case of a patient with perimembranous small ventricular septal defect and infundibular stenosis suffered from pulmonary valve endocarditis and septic pulmonary embolism.     

Periarterial abscess: another cause of cyanosis after pulmonary artery banding

Twenty seven months after pulmonary artery banding a boy aged two and a half developed rapidly progressive cyanosis. A periarterial abscess was found at the site of the band. Repair of the pulmonary artery and closure of the ventricular septal defect were complicated by profoundly low cardiac output and prolonged dependence on a ventilator. This near fatal complication would have been avoided by primary closure of the ventricular septal defect.