Tricuspid atresia, hypoplastic right ventricle, intact ventricular septum and congenital absence of the pulmonary valve.

A case of tricuspid atresia by sealing of the valves, associated with an intact ventricular septum, a hypoplastic right ventricle and the absence of the pulmonary valve, is described. The foramen ovale and the ductus arteriosus were widely patent but there was no interventricular communication. The pulmonary flow was assured by the ductus which had a saccular continuation in the pulmonary trunk. It is postulated that the lesions probably occurred during the period between the XIX and XXII horizons and that the malformed right coronary artery may have played an important role in producing the lesions of the right ventricle.     

Uhl's anomaly associated with atresia of the pulmonary valve

A case of Uhl's anomaly in a newborn who died in congestive cardiac failure at 18 days of age is described. There was severe cardiomegaly on chest X-ray, right atrial hypertrophy on E.C.G., and a delayed tricuspid valve closure of 0,8 seconds on M-mode echocardiogram. The necropsy study showed a huge right atrial and right ventricular dilatation, a very thin right ventricular wall (1 mm.) with an important reduction in the number of muscular fibers, a pulmonary valve atresia, and a patent ductus arteriosus and foramen ovale. We have only three cases Uhl's anomaly associated with pulmonary atresia, and no one with such an important delay of the tricuspid valve closure on the echocardiogram.     

Critical Ebstein Anomaly in a Fetus Successfully Managed by Elective Preterm Delivery and Surgical Intervention Without Delay After Birth

This report describes a case of Ebstein anomaly in a fetus with cardiomegaly, severe tricuspid regurgitation, pulmonary regurgitation, and retrograde ductal flow that showed a marked increase in the size of the right atrium with advancing gestational age. Elective preterm delivery was performed at 35 weeks gestation. The prostaglandin E1 infusion resulted in more pronounced systemic hypotension and acidosis secondary to circular shunt across the patent ductus arteriosus as well as pulmonary regurgitation and tricuspid regurgitation. Emergency surgical intervention consisting of main pulmonary artery ligation, ductus arteriosus ligation, central shunt creation, and plication of the right atrium without cardiopulmonary bypass was performed 4 h after birth. At the age of 16 days, the Starnes procedure was performed. The infant’s postoperative course was uneventful. A fetus that has Ebstein anomaly associated with pulmonary regurgitation is at risk for circular shunt across the patent ductus arteriosus after delivery. Planned delivery and surgical intervention without delay after birth are useful for the treatment of such cases.     

Coarctation of the aorta in the syndrome of absent pulmonary valve with ventricular septal defect

Summary The case of a male child is reported in which the syndrome of absent pulmonary valve and ventricular septal defect was associated with anomalous drainage of the right superior pulmonary vein into the superior vena cava, atrial septal defect, a right aortic arch, and an aortic isthmic coarctation. The combination of aortic coarctation with a high degree of right-ventricular outflow obstruction has not been reported so far in the literature. This case shows that there is no proof of aplasia of the ductus arteriosus in the syndrome of absent pulmonary valve and ventricular septal defect. Most likely, there is an early involution of the ductus arteriosus; persistence of the ductus arteriosus would not be compatible with fetal survival.     

Large patent ductus arteriosus and interventricular communication associated with congenital absence of the pulmonary valve

A case of pulmonary valve agenesis with ventricular septal defect and large patent ductus arteriosus is reported in a neonate. Heart failure occurred at 10 days of life; clinical examination showed continuous murmur. Echocardiography and angiocardiography visualized a major dilatation of the pulmonary artery. At cardiac catheterization there was an important left to right shunt through a large ductus, and pulmonary hypertension. There was also marked pulmonic regurgitation. Because of respiratory distress, ligation of ductus arteriosus was performed at 5 weeks of life. Five years later the patient is still in good condition. Patent ductus arterious is rarely associated with pulmonary valvular agenesis and ventricular septal defect. Our case is not explained by the fetal circulation theory described in this heart malformation.     

Surgical closure of the tricuspid valve for pulmonary atresia,intact ventricular septum,and right ventricle to coronary artery communications

Summary A surgical approach is reported for a patient with pulmonary atresia, intact ventricular septum, and right ventricle to coronary artery communications through sinusoids. A shunt procedure was performed at two days of age; the right ventricular outflow tract was not opened. At subsequent catheterization, the tricuspid valve was temporarily closed with a balloon catheter and no change was seen in the ECG. At five months of age, the right ventricle was plicated and a patch was sewn over the tricuspid valve. One year after surgery, neither the right ventricular cavity nor the sinusoids could be demonstrated at angiocardiography; ECG changes of left ventricular ischemia have resolved, and the child is growing normally.     

Persistent truncus arteriosus: Pathologic anatomy in 54 cases

Summary Fifty-four specimens of heart with persistent truncus arteriosus (PTA) were reviewed anatomically. According to the Collett-Edwards classification [11] there were 28 examples of type I and 26 type II. The sex distribution was equal. The number of the truncal cusps ranged from one to four (42% tricuspid, 30% bicuspid, 24% quadricuspid, and 4% unicommissural). A unicommissural truncal valve has not been previously reported. In 72% of cases, the truncal valve leaflets were thickened or dysplastic. Two valves were stenotic. The truncus arteriosus originated from both ventricles equally in 42% of the cases, predominantly from the right ventricle in 42%, and predominantly from the left ventricle in 16% of the cases. In unoperated cases of PTA originating predominantly from the right ventricle, it appeared to us that usual operative correction might result in left ventricular outflow obstruction. Variations in coronary arterial origins and patterns were present in nearly half of the cases. A single coronary artery was observed in ten cases (18.5%). Stenosis of the ostium of one coronary artery was seen in each of four cases (7%). High posterior origin of the left coronary artery was observed in ten cases (18.5%). Among the associated cardiovascular anomalies, the most common were right aortic arch (36%) and interruption of the aortic arch (11%). Three cases with the latter condition exhibited crossed pulmonary arteries. Isolated cases with tricuspid atresia, vascular sling (left pulmonary artery arising from right pulmonary artery), and persistent common atrioventricular canal were encountered.     

A case of severe Ebstein's anomaly with incompetent pulmonary valve

A case of Ebstein's anomaly with functional pulmonary atresia diagnosed in utero is presented. The diagnosis was confirmed by postnatal echocardiographic, angiographic, and postmortem pathologic findings. On echocardiography the septal leaflet of the tricuspid valve was displaced towards the right ventricular apex. The tricuspid valve was moderately regurgitant and the arterial duct was patent. Continuous wave or color Doppler revealed serious reduction in forward flow from right ventricle through the pulmonary arteries; however, massive pulmonary regurgitation was observed. Pulmonary circulation was dependent on the ductal flow due to functional pulmonary atresia. Angiography revealed the massively enlarged right atrium, the absence of forward flow through the tricuspid valve, transfer of contrast material through the atrial septal defect to the left atrium, and the retrograde inflow of the pulmonary arteries from the aorta via the patent arterial duct. Ebstein's anomaly accompanied by functional pulmonary atresia is very rare. The fetal and neonatal presentation of this anomaly is associated with poor outcome.     

Echocardiographic findings of pulmonary atresia or critical pulmonary stenosis and intact ventricular septum in utero

BACKGROUND: Neonates with right ventricular outflow obstruction and intact ventricular septum show serious hemodynamic problems, such as severe hypoxemia, congestive heart failure due to massive tricuspid regurgitation, respiratory distress related to huge pulmonary hypoplasia or ventricular dysfunction due to right ventricle-coronary communication. Recent advances in fetal diagnosis include many cases of in utero diagnosis of pulmonary atresia or critical pulmonary stenosis and intact ventricular septum. METHOD: Among the fetuses examined from April 1994 to March 1998, five fetuses were found with pulmonary atresia (PA) or critical pulmonary stenosis (CPS). Fetal echocardiograms were reviewed to elucidate the accuracy of fetal information and the efficacy of fetal diagnosis in the perinatal management of patient with CPS or PA and intact ventricular septum. RESULTS: The five cases were divided into two groups: two with a very small right ventricle (group 1) and three with a tripartite right ventricle (group 2). Fetal cardiomegaly and right atrial dilatation were prominent in group 2, whereas cardiac sizes were normal in group 1. Serial fetal examination in one group 2 fetus revealed developing right ventricular hypertrophy in utero. All group 2 cases showed massive tricuspid regurgitation (TR). Estimated right ventricular pressures from TR always exceeded the systemic blood pressures of gestational age-matched neonates. Reversed flow through the ductus arteriosus was recorded in both groups and ductus-dependent pulmonary circulation after birth was anticipated. Patency of both tricuspid and pulmonary valves was difficult to recognize in utero, as was right ventricle-coronary artery communication. Four of the five cases were maternally transported and survived palliative and/or definitive intervention. One fetus with chromosomal abnormality was observed without intervention during infancy and received palliative surgery when she was two years old. CONCLUSION: Fetal hemodynamic information was useful for making decisions not only after birth, but also in utero, and may eventually result in improving the prognosis of babies with PA/CPS. Serial observation of the fetuses with PA/CPS may also suggest the possible pathogenesis of PA/CPS in utero.     

经导管射频打孔并球囊扩张治疗婴儿室间隔完整的肺动脉瓣闭锁

目的探讨经导管射频瓣膜打孔并球囊扩张治疗婴儿室间隔完整的肺动脉瓣闭锁（PA／IVS）有效性和安全性。方法4例患儿年龄分别为11、9、9个月和12d,体重4—10kg[（7．6±2．2）kg]。均具有紫绀和动脉导管未闭连续性杂音的临床表现。超声心动图诊断后,在全麻下作右心室造影确定右心室腔由3部分结构组成,漏斗部呈长管形,无冠状动脉异常。适合经导管射频打孔并球囊扩张术治疗。射频瓣膜打孔能量5—8W,持续时间2—5s,一般2次;预扩球囊3mm×20mm-5mm×20mm,6—14atm;肺动脉瓣扩张球囊10mm×30mm-14mm×30mm,有效扩张1-2次。手术持续时间120—150min,X线暴露时间25．4—43．9min。结果技术成功率100％,除1例新生儿发生心脏穿孔早期死亡外,其他3例无任何并发症。存活3例随访4-12个月,1例需外科解除右室流出道梗阻,2例可择期封堵动脉导管未闭。结论经导管射频瓣膜打孔并球囊扩张术治疗PA／IVS需严格掌握适应证,规范操作,可作为替代或延迟外科开胸手术治疗的一种方法。     

Tricuspid atresia with imperforate valve: Angiographic findings and surgical implications in two cases with AV concordance and normally related great arteries

Summary The angiographic and intraoperative findings are described in two children with imperforate tricuspid valve with atrioventricular and ventriculoarterial concordance who underwent a successful Fontan operation. It is important from a surgical point of view to distinguish this rare variant of tricuspid atresia from the more classic form with absent atrioventricular connection. The characteristic feature of hearts with imperforate tricuspid valve is the presence of a normally formed right ventricle which, in the presence of ventriculo-arterial concordance, can be used to support the pulmonary circulation. When the imperforate valve is large, it may be possible to remove it, and restore atrioventricular continuity. Alternatively, an atrioventricular type of Fontan repair using a valved conduit should be considered as the treatment of choice in the presence of a suitable pulmonary arterial tree and pulmonary valve.     

Comparison of effects of 95% and 90% oxygen saturations in respiratory distress syndrome.

AIMS: To determine if decreasing arterial blood saturation from 95% to 90% could cause vasoconstriction of the pulmonary vasculature and dilatation of a patent ductus arteriosus in preterm newborn infants with respiratory distress syndrome (RDS). METHODS: Doppler echocardiographic studies were compared at 95% and 90% pulse oxygen saturation (SpO2) in 13 preterm infants aged 61.7 (4.3) hours with RDS and Doppler echocardiographic evidence of tricuspid regurgitation. RESULTS: The mean (SD)Doppler echocardiographic indices determined at 95% were heart rate (146 (3.60) beats per minute), acceleration time of the velocity wave forms of the pulmonary artery (PAAT) (51.8 (2.5) milliseconds), ratio of PAAT to right ventricular ejection time (ET) (0.26 (0.02)), diameter of the ductus arteriosus (2.6 (0.6) mm), pulmonary blood flow (0.33 (0.03) l/minute) and the left ventricular shortening fraction (SF)(0.4 (0.02)%). The ascending aorta flow velocity wave form was used for the calculation of pulmonary blood flow. The right ventricular to right atrial systolic pressure gradient calculated using the peak velocity of the tricuspid regurgitation jet was 26.7 (7.4) mm Hg. CONCLUSIONS: A decrease from 95% to 90% SpO2 did not have any effect on the pulmonary circulatory haemodynamics nor the ductus arteriosus.     

Angiographic evidence of absent ductus arteriosus in severe right ventricular outflow obstruction

Summary The angiocardiograms of 5 newborn infants with autopsy and/or surgically-proven congenital absence of the ductus arteriosus (ADA) and right ventricular outflow obstruction (Group A), and of 14 neonates with pulmonary atresia complex and patent ductus arteriosus (Group B) were reviewed. Aortic size was similar in both groups; however, the diameters of the right and left pulmonary arteries were much smaller in Group A than in Group B (right pulmonary artery: 2.6 vs 4.5 mm,P<0.005; left pulmonary artery: 2.5 vs 4.3 mm,P<0.005). Extensive bronchial collaterals were observed in Group A but not in Group B. Tricuspid aortic valve stenosis was present in 2 patients in Group A but in none in Group B. The diagnosis of ADA may be made in newborn infants with severe right ventricular outflow obstruction if the angiocardiograms reveal hypoplasia of the pulmonary arteries, extensive bronchial collaterals, and nonvisualization of the ductus arteriosus. Other suggestive features include aortic valve stenosis and/or right aortic arch with aberrant left subclavian artery.Supported in part by Grant RR-305 from the General Clinical Research Centers Program of the Division of Research Resources and by Grant HL-07605 from the National Heart, Lung and Blood Institute. National Institutes of Health, Bethesda, Maryland     

Rudimentary pulmonary valve and ventricular septal defect associated with patent ductus arteriosus

This report describes a rare case of patency of the ductus arteriosus in the setting of ventricular septal defect and rudimentary pulmonary valve. We comment on the fetal, neonatal, and postmortem findings and speculate about the role of the ductus arteriosus in the development of dilatation of the pulmonary arteries in this malformation.     

“Absent” pulmonary valve with atrial septal defect and patent ductus arteriosus

Summary The case of a newborn with absent pulmonary valve, atrial septal defect, and patent ductus arteriosus is reported. He underwent surgical repair at five weeks of age, with closure of the patent ductus arteriosus and porcine pulmonary valve insertion. The clinical course leads us to suggest that reduction of pulmonary artery pressure and pulmonary valve competence will lead to prompt improvement in tracheobronchial compression and respiratory symptoms; no pulmonary arterioplasty is needed.     

Aplasia of semilunar valve leaflets: Two case reports and developmental aspects

Summary Two fetuses of approximately 11 weeks development with aplasia of the pulmonary as well as the aortic valve leaflets are reported. Both cases showed additional cardiac malformations. Case 1, with all leaflets missing, also had double-outlet right ventricle, hypplastic left ventricle, large ventricular septal defect, straddling tricuspid valve, and atretic mitral valve. Case 2, with only one hypoplastic aortic valve leaflet, showed hypoplasia of the mitral valve and the left ventricle, and a subaortic ventricular septal defect.The observations made and data in the literature suggest that aplasia of semilunar valve leaflets reflects an underdevelopment of the endocardial cushion swellings at the ventriculoarterial junction, rather than resulting primarily from a malseptation of the cardiac outflow tract.     

经右室穿刺肺动脉瓣球囊扩张治疗室间隔完整型肺动脉闭锁

目的 评价经右心室穿刺肺动脉瓣球囊扩张杂交手术治疗室间隔完整型肺动脉闭锁的早期治疗结果.方法 6例室间隔完整型肺动脉闭锁患儿接受右心室穿刺肺动脉瓣球囊扩张杂交手术.术前平均经皮氧饱和度(71±10)%.结果 全组无死亡及围术期并发症发生.术后经皮氧饱和度为(89±5)%;右室-肺动脉压差为(19±11)mm Hg.随访(4±3.6)个月,跨肺动脉瓣压差(45±42)mm Hg,1例术后再次行经导管肺动脉瓣球囊扩张.结论 对于右心室发育良好的室间隔完整型肺动脉闭锁,本术式早期疗效满意,安全性较高,术后肺动脉瓣反流少,可以促进右心室的发育.

Abstract：

Objective To evaluate the early outcomes of balloon valvuloplasty via right ventricle pulmonary valve for the treatment of pulmonary atresia with intact ventricular septum in children.Methods Methods Between January 2010 and December 2010, 6 patients were diagnosed with pulmonary atresia with intact ventricular septum, and underwent balloon valvuloplasty via right ventricle pulmonary valve at this center. Of the 6 patients, the mean body weight was 3. 8 ± 1.6 kg, the mean transcutaneous oxygen saturation was 71 ± 10%, and the mean age was 56 ± 45 days. All of the 6 patients had tripartite right ventricles. Their tricuspid valve z-scores ranged from -2 to 2. 2 (mean zscore: 0. 4 ± 1. 5). Results During operation, the mean size of the balloons used was 7. 3 ± 1. 2 mm,the ratio of the balloon size to pulmonary valve ring was 1.0 ± 0. 1, the pressure gradient between right ventricle and main pulmonary artery was 19 ± 11 mmHg, the ratio of forward flow from right ventricle to that from ductus arteriosus was 0. 35 ± 0. 06. After surgery, the mean ventilation time was 3. 2 ± 1.0 days, and mean ICU stay was 16 ± 5 days. The post-operative transcutaneous oxygen saturation was 89 ± 5%. The patients were followed up for 4 ± 3. 6 months. No death and post-operative complications were noted. One patient underwent interventional balloon valvuloplasty 2 weeks after the first surgery. The transcutaneous oxygen saturation on last follow-up was 85 ± 6%, which was significantly improved after surgery (t = -2. 66, p = 0. 045). The postoperative pressure gradient between right ventricle and main pulmonary artery was 45 ± 42 mmHg. Four of the 6 patients had pulmonary regurgitation, 3 of whom were mild and 1 was moderate. Tricuspid valve z-scores (mean, 0. 5± 1) also improved compared with that before procedure (t = -3. 36, p = 0. 02). Conclusions Balloon valvuloplasty via right ventricle pulmonary valve is safe and effective to treat the pulmonary atresia with intact ventricle septum in children.     

Balloon Dilatation of the Pulmonary Valve in a Patient with Pulmonary Atresia and Intact Ventricular Septum Using a Commercially Available Radiofrequency Catheter

Using a commercially available 5F deflectable radiofrequency catheter, we have succeeded in percutaneous valvotomy of an imperforate pulmonary valve and consecutive balloon dilatation in a baby with pulmonary atresia and intact ventricular septum. After the procedure, right ventricular systolic pressure fell from 125 mmHg to 65 mmHg, and right ventriculography demonstrated anterograde blood flow into the pulmonary arteries. There were no major complications. Doppler echocardiography at 1 year after the procedure demonstrated a pressure gradient across the pulmonary valve of 20 mmHg with mild pulmonary and tricuspid regurgitations.     

Absent Pulmonary Valve with Intact Ventricular Septum Presenting as Cardiorespiratory Failure at Birth

Absent pulmonary valve with intact ventricular septum is a rare congenital anomaly. Most cases are not diagnosed until years after birth, although several cases have been reported in the literature in which cardiorespiratory distress developed during the neonatal period. We present a patient who showed improvement after fairly conservative therapy to alleviate the pulmonary vascular resistance, with ultimate palliative surgery. The aneurysmal dilatation of the pulmonary artery was for the most part confined to the proximal portion of the trunk, possibly because the ductus arteriosus was patent. The literature is reviewed.     

Absent Pulmonary Valve with Tricuspid Atresia or Severe Tricuspid Stenosis: Report of Three Cases and Review of the Literature

Absence of the pulmonary valve occurs usually in association with tetralogy of Fallot and occasionally with an atrial septal defect or as an isolated lesion. Very rarely it occurs with tricuspid atresia, intact ventricular septum, and dysplasia of the right ventricular free wall and of the ventricular septum. We present the clinical, anatomic, and histologic findings of a new case, and for the first time, the data from two patients with absent pulmonary valve and severe tricuspid stenosis, who exhibited similar histologic findings. We also reviewed the clinical and anatomic data of 24 previously published cases and compared them with the new cases. In all three new cases, the myocardium of the right ventricle was very abnormal. In the two cases with tricuspid stenosis, large segments of myocardium were replaced with sinusoids and fibrous tissue. In the case with tricuspid atresia, the right ventricular free wall contained only fibroelastic tissue. The ventricular septum in all three patients showed asymmetric hypertrophy and in two of the three patients, multiple sinusoids had replaced large segments of myocardial cells. The left ventricular free wall myocardium and the walls of the great arteries were unremarkable. Our data indicate that myocardial depletion involving the right ventricular free wall and the ventricular septum and its replacement by sinusoids and fibroelastic tissue occur not only in cases of absent pulmonary valve with tricuspid atresia but also in cases of absent pulmonary valve with tricuspid stenosis. The degree of myocardial depletion varies and is more severe when the tricuspid valve is atretic. Received April 8, 1999; accepted July 12, 1999.