Management of pulmonary atresia or critical pulmonary stenosis and intact ventricular septum with a small or hypoplastic right ventricle

Twenty-one neonates and infants less than 3 months old undergoing cardiac surgery for an obstructed right ventricular outflow tract, intact ventricular septum and a small or hypoplastic right ventricle were retrospectively analyzed, in order to assess the effects of a change in management protocol. Seven of the 8 patients with critical pulmonary stenosis survived surgery using a transannular outflow patch, whereas only 1 of the 8 patients with pulmonary atresia survived the same operation. Two patients in the latter group died 2 and 3 months after surgery but with complications arising from surgery. Of 5 patients with pulmonary atresia who had a modified Blalock Taussig shunt, 3 patients survived the surgery and were discharged home. These results significantly indicate that there is an unacceptably high mortality for the relief of pulmonary atresia (with intact septum) using a transannular outflow patch, and a Blalock Taussig shunt is the preferred operation. The transannular outflow patch is a safe operation for neonates with critical pulmonary stenosis, irrespective of the size of the right ventricle.     

Abnormalities of the coronary circulation in pulmonary atresia and intact ventricular septum

To identify developmental aspects and establish morphologic criteria useful for clinical recognition of sinusoids and coronary artery abnormalities in pulmonary atresia and intact ventricular septum, we studied 17 autopsy hearts with this lesion. Hearts from age- and weight-matched infants served as controls. There were two stillborn fetuses, 12 full-term neonates, and 3 infants. None of the eight hearts with normal or large-sized right ventricular chambers had sinusoids or coronary artery abnormalities. Five of nine hearts with small right ventricular chambers had sinusoids; all five lacked trabecular and outlet portions of the ventricle. Four of these five contained major coronary artery abnormalities: atrophy of one or both coronaries proximal to a communication between a sinusoid and the coronary artery. We conclude (1) there is a strong association between the presence of sinusoids and coronary anomalies, supporting the developmental concept of abnormal persistence of embryonic right ventricular sinusoid--coronary artery communications in pulmonary atresia and intact ventricular septum; (2) the absence of trabecular and outlet portions of the small right ventricle indicates a strong possibility of coronary artery abnormalities; and (3) this initial report of a fully-developed lesion in a mid-gestation fetus documents that the coronary artery problem may arise early in fetal life.     

Successful transcatheter coil embolization of coronary artery to left ventricular fistula associated with absent pulmonary valve with tricuspid atresia in early infancy.

Transcatheter coil embolization for coronary artery to left ventricular fistula was successfully performed in a neonate. At 30 weeks' gestation, fetal echocardiography showed a hypoplastic right ventricle with intact ventricular septum, absent pulmonary valve, tricuspid atresia, and marked distension of the right coronary artery. After birth, the neonate had congestive heart failure and the electrocardiogram showed myocardial ischemic changes in the left ventricular area. Aortography showed a dilated right coronary artery arising from the ascending aorta and draining into the left ventricle. Transcatheter coil embolization was carried out on the 9th day after birth. Since the procedure, no myocardial ischemic changes have been detected. Transcatheter coil embolization is a useful therapy for coronary artery fistula associated with myocardial ischemia.     

Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum diagnosed during fetal life

OBJECTIVES: To establish the prevalence of coronary arterial abnormalities in mid-trimester fetuses with pulmonary atresia with intact ventricular septum, and whether their presence correlates with right ventricular morphology. BACKGROUND: The presence of coronary arterial fistulas significantly alters the surgical options and prognosis for patients with pulmonary atresia with intact ventricular septum. The lesion can reliably be diagnosed using fetal echocardiography, and further definition of the prognosis is important for counselling parents. METHODS: We examined the hearts of 39 pathological specimens diagnosed during fetal life, 3 of whom died postnatally. Coronary arterial abnormalities were defined as non-connection of the left or right coronary arteries to the aorta, ostial stenosis, marked tortuosity, dilation, thickening or abnormal myocardial branching. Mild tortuosity, or myocardial bridging, were considered normal. We measured the dimensions of the tricuspid valve along with the inlet and outlet portions of the ventricles. Ebstein's malformation, tricuspid valvar dysplasia, and the presence or absence of the infundibulum, were especially noted. We examined also 12 normal hearts as controls. RESULTS: Coronary arterial abnormalities were found in 14/39 (36%). The dimensions of the right ventricle and tricuspid valves, and the gestational ages of the fetuses, were compared for these 14 with the 25 having no abnormalities using independent t-tests. The gestational ages were similar, 21.9 vs 21.1 weeks. The mean dimensions of the tricuspid valve, median z-scores, and right ventricle were smaller, 2.9 vs 7.2 mm; p < 0.002; -4.46 vs 0.23; p < 0.03; and 6.9 vs 13.7 mm; p < 0.002, for those with coronary arterial abnormalities. Ebstein's malformation, or dysplasia of the tricuspid valve, was present in 4 of 14 with, vs 15 of 25 without, coronary arterial abnormalities. A patent infundibulum was noted in 34 of 39 specimens. CONCLUSIONS: Mid-trimester fetuses with pulmonary atresia with intact ventricular septum already exhibit coronary arterial abnormalities, with a prevalence of 36%. The presence of a patent infundibulum confirms that atresia of the pulmonary valve is an acquired process. Coronary arterial abnormalities are seen in 50% of those with hypoplastic right ventricles, but less frequently in the presence of well developed ventricles. This is important information for those involved in counselling parents.     

Cardiac Computed Tomography of Multiple Coronary Arteries to Right Ventricle Fistulas in a Newborn With Pulmonary Atresia and Intact Ventricular Septum

Right ventricle-dependent coronary circulation significantly alters the therapeutic options and prognosis in neonates with pulmonary atresia and intact ventricular septum. The contribution of cardiac computed tomography for the accurate assessment of multiple coronary arteries to right ventricle fistulas in a newborn infant with pulmonary atresia and intact ventricular septum is described.     

Pulmonary atresia with an intact interventricular septum and Ebstein's anomaly of the tricuspid valve

Patients with pulmonary atresia and intact ventricular septum have a poor prognosis with or without conventional surgical treatment. The best results of surgical treatment are obtained in those cases with a mild underdeveloped right ventricle and minor sinusoidal communication in the absence of important dysfunction of the tricuspid valve. We present five cases of pulmonary atresia with intact ventricular septum associated with dysfunction of the tricuspid valve. On the basis of radiographic, electrocardiographic and hemodynamic findings, this group of patients could not be distinguished from others without dysplasia of the tricuspid valve. Echocardiographic and angiocardiographic studies are mandatory in the differential diagnosis. A combination of systemic-pulmonary artery anastomosis associated with pulmonary valvotomy, when possible, and reconstruction of the right ventricular outflow tract are indicated for surgical solution of these malformations. However, tricuspid valve replacement is indicated in some cases.     

Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum.

BACKGROUND. Coronary artery anomalies including 1) right ventricle (RV)-to-coronary artery fistulas, 2) coronary artery stenoses, and 3) coronary occlusions occur in patients with pulmonary atresia with intact ventricular septum (PA-IVS). In some, a large part of the coronary blood supply may depend on the RV. This RV-dependent coronary circulation may determine survival after right ventricular decompression (RVD): RVD may cause RV "steal" in the presence of fistulas alone and ischemia, coronary isolation, or myocardial infarction in the presence of coronary stenoses. METHODS AND RESULTS. Eighty-two patients with PA-IVS who presented between January 1979 and January 1990 were reviewed; 26 (32%) had RV-to-coronary artery fistulas. Of these 26, 23 had adequate preoperative coronary angiograms for analysis. RVD was achieved in 16. Seven of 16 had fistulas only; each survived RVD. Six of 16 had stenosis of a single coronary artery [left anterior descending coronary artery (LAD), four; right coronary artery (RCA), two]; four of six survived RVD. Three of 16 had stenoses and/or occlusion of both the RCA and LAD; all three died shortly after RVD of acute left ventricular dysfunction. CONCLUSIONS. 1) Potential RV steal alone does not preclude successful RVD. 2) Fistulas with stenoses to a single coronary artery may not preclude successful RVD. 3) RVD appears to be contraindicated in the presence of stenoses and/or occlusion involving both the right and left coronary systems. Nonsurvival after RVD seems to depend on the amount of the left ventricular myocardium at risk, i.e., that which is distal to coronary artery stenoses, especially when involvement of both coronary arteries limits effective collateralization. Precise definition of coronary arterial anatomy is mandatory in neonates with PA-IVS.     

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.     

Severe tricuspid stenosis presenting as tricuspid atresia. Echocargraphic diagnosis and surgical management.

Two cases of pulmonary atresia with intact ventricular septum and severe tricuspid stenosis are described in which the initial angiographic diagnosis was tricuspid atresia and pulmonary atresia. Two dimensional echocardiography showed the features of an imperforate tricuspid valve because in each case contrast echocardiography failed to show anterograde flow from the right atrium to right ventricle. Successful radical surgical repair was achieved in one patient with performing a tricuspid valvotomy and inserting an external valved conduit between the hypoplastic right ventricle and the main pulmonary artery. In the second case, an infant died four weeks after tricuspid valvotomy and right ventricular outflow tract reconstruction.     

Laser-assisted tricuspid valve balloon dilation for acquired tricuspid valve atresia.

Acquired pulmonary valve atresia is a well-recognized but uncommon complication of surgical systemic-to-pulmonary artery shunts in patients with tetralogy of Fallot. Acquired atresia of the tricuspid valve, however, has not been reported previously. This complication developed in a 3-year-old girl, with pulmonary atresia and an intact ventricular septum, after a Blalock-Taussig shunt and right ventricular outflow tract reconstruction. Percutaneous transcatheter laser-assisted balloon dilation re-established antegrade flow across the tricuspid valve.     

Three anomalies of the coronary arteries co-existing in a case of pulmonary atresia with intact ventricular septum

A female infant, who died 17 hours after spontaneous birth, was found to have three distinct coronary arterial anomalies in association with pulmonary atresia with intact ventricular septum. These anomalies were first, an aneurysmal left coronary artery with a fistulous communication into the right ventricle; second, an anomalous origin of the right coronary artery from the pulmonary artery; and third, a supernumerary coronary artery arising from the right ventricle. Although the coronary arterial system was entirely connected to the right ventricle, the perfusing blood originated from the left ventricle and there was no clinical or histological evidence of significant myocardial ischaemia.     

Right ventricular infarction with cardiac rupture in an infant with pulmonary valve atresia with intact ventricular septum

This report describes an 8 day old infant with pulmonary valve atresia, hypoplastic and hypertensive right ventricle and myocardial sinusoid-left anterior descending coronary artery connections. A large right ventricular sinusoid complex developed that was associated with transmural right ventricular necrosis and cardiac rupture. This is the first report to document transmural myocardial infarction and rupture in pulmonary valve atresia with intact septum, and this condition should be added to the causes of myocardial infarction in infancy. A reduced number of caliber of sinusoid-coronary artery channels may be responsible for right ventricular damage in this condition.     

Tricuspid atresia: analysis of coronary artery distribution and ventricular morphology.

There is still disagreement concerning the precise nature of the anterior ventricular chamber in "tricuspid atresia". Some argue that it is a right ventricle, while our own previous studies have suggested it is comparable to the outlet chamber seen in classical "single ventricle". We have compared the morphology of the anterior ventricular chambers in 48 examples of tricuspid atresia (absent right atrioventricular connection), 24 hearts with double inlet to the left ventricular chamber, and 15 hearts with pulmonary atresia and intact ventricular septum. Since there is further disagreement concerning the nature of the coronary artery which delimits the posterior extent of the ventricular septum in these hearts, we analysed the position of this vessel relative to external reference points on the atrioventricular junction. No significant difference was found with respect to the morphometry of hearts with tricuspid atresia (absent right atrioventricular connection) and those with double inlet. In both groups, however, significant differences were shown between hearts with ventriculoarterial concordance and discordance. Coronary artery disposition was the same in both groups and different from that found in hearts with pulmonary atresia and intact septum. We conclude that the ventricular morphology is comparable in hearts with tricuspid atresia (absent right atrioventricular connection) and those with double inlet to a left ventricular chamber. The lesions are distinguishable by their atrioventricular connection which is nevertheless univentricular in both.     

Muscular Infundibular Atresia Is Associated with Coronary Ostial Atresia in Pulmonary Atresia with Intact Ventricular Septum

Objective. To determine whether infundibular morphology is associated with coronary ostial atresia and/or right ventricle dependent coronary circulation in patients with pulmonary atresia with intact ventricular septum. Design. Neonatal echocardiograms of patients with pulmonary atresia with intact ventricular septum were evaluated for tricuspid valve size and infundibular anatomy (muscular pulmonary atresia vs. membranous pulmonary atresia). Right ventricle dependent coronary circulation and coronary ostial atresia were diagnosed angiographically. Medical record review determined patient outcome. Setting. Tertiary‐care pediatric hospital. Results. Of 72 patients, 24 had muscular pulmonary atresia including 23 with right ventricle dependent coronary circulation. Fourteen of these had unilateral or bilateral coronary ostial atresia. Of 46 patients with membranous pulmonary atresia, 5 five had right ventricle dependent coronary circulation and none had coronary ostial atresia. Muscular pulmonary atresia patients had smaller tricuspid valve z‐scores and were less likely to achieve a biventricular repair than those with membranous pulmonary atresia (P < .01). Muscular pulmonary atresia had 82% sensitivity, 98% specificity, and 96% positive predictive value for presence of right ventricle dependent coronary circulation. In the group with membranous pulmonary atresia, there were two deaths, no transplants, and 23 (48%) achieved a biventricular repair. In contrast, of the 24 with muscular pulmonary atresia, there were seven deaths, two transplants, and no biventricular repairs. Conclusions. In this cohort, muscular pulmonary atresia was strongly associated with right ventricle dependent coronary circulation and coronary ostial atresia, and appears to be a useful morphologic marker for poor outcome among pulmonary atresia with intact ventricular septum patients. This information may be useful during prenatal counseling and for presurgical evaluation.     

Angiojet catheter-based thrombectomy in a neonate with postoperative pulmonary embolism.

A 2-day-old male with pulmonary atresia and an intact ventricular septum underwent central aortopulmonary shunt placement and right ventricular outflow tract reconstruction. Postoperatively, the patient had difficulty weaning from mechanical ventilation and was found to have a large thrombus occluding the entire right lower pulmonary artery. The patient underwent successful percutaneous catheter-based thrombectomy in the early postoperative period with the Possis AngioJet catheter. The patient had immediate improvement in gas exchange and was able to be weaned from the ventilator shortly thereafter. This represents the first published use of mechanical thrombectomy in a postoperative neonate.     

Vascular aneurysm producing divided right atrium in a patient with pulmonary atresia and intact ventricular septum

We describe a patient with pulmonary atresia and intact ventricular septum in whom the right atrium was divided by a vascular aneurysm located in the right atrioventricular groove. We postulate that the structure represents an aneursymally dilated right coronary artery taking anomalous origin from the pulmonary trunk, with fistulous communication to the right atrium. We discuss the findings relative to concepts of development of the coronary arteries in normal hearts and in pulmonary atresia with an intact ventricular septum.     

Pulmonary atresia with intact ventricular septum and antenatal left ventricular failure

Pulmonary atresia with intact ventricular septum has been described extensively, and it is recognized that associated abnormalities of the coronary arteries may cause ischemia. We describe a fetus, diagnosed antenatally as having pulmonary atresia with intact ventricular septum and severe hypoplasia of the right ventricle, who developed severe left ventricular dysfunction. We hypothesize that this is due to reduced coronary blood flow because of falling right ventricular volume in the presence of complete right ventricular coronary arterial dependence.     

Recognition of abnormal connections of coronary arteries with the use of Doppler color flow mapping

Anomalous connection of a coronary artery to a ventricle or pulmonary artery causes shunting of blood from the coronary circuit and may produce myocardial ischemia. Such a coronary anomaly may occur in isolation or with other defects. Doppler color flow mapping and two-dimensional echocardiography were used to diagnose anomalous coronary connections in 13 patients, 1 day to 7 years of age, over a 1 year period. The diagnoses were anomalous origin of the left coronary artery from the pulmonary trunk in five patients, a coronary artery to left ventricle fistula or coronary artery to pulmonary artery fistula in four patients with other complex defects, right ventricular sinusoids in two patients with pulmonary atresia and intact ventricular septum and an isolated coronary artery fistula in two patients. In all cases, the abnormal coronary connection was recognized on the basis of an abnormal, continuous or to and fro flow pattern in the fistula and its connections as demonstrated by scanning in multiple views with Doppler color flow mapping. The low spatial resolution of Doppler color flow mapping limits the anatomic detail available; nonetheless, it is a significant advance in the noninvasive diagnosis of abnormal coronary connections.     

Two-dimensional echocardiographic evaluation of right ventricular size and function in newborns with severe right ventricular outflow tract obstruction

Critical pulmonary stenosis or atresia with intact ventricular septum represents a congenital cardiac lesion for which the long-term prognosis appears to depend partly on the size of the right ventricle. Thus, the capability of noninvasive assessment of right ventricular size to predict operative outcome was examined in 15 infants (aged 1 to 30 days, mean 5.6) with severe right ventricular outflow tract obstruction (either critical pulmonary stenosis [7 patients] or pulmonary atresia with intact ventricular septum [8 patients]). Using echocardiography in two orthogonal subxiphoid views, right ventricular volume, wall thickness, area change fraction, ejection fraction and tricuspid anulus dimension were measured. All patients with a normalized right ventricular enddiastolic volume of less than 5 ml/m2 and a normalized tricuspid anulus dimension of less than 1.0 cm/m2/3 required a shunt operation. Only one patient with a volume of more than 6 ml/m2 and a normalized tricuspid anulus dimension of more than 1.4 cm/m2/3 required more than relief of right ventricular outflow tract obstruction. In this patient, residual severe pulmonary stenosis necessitated the shunt procedure. One patient with a volume of more than 6 ml/m2 had an anulus diameter of less than 1.4 cm/m2/3 and one patient with an anulus diameter of more than 1.4 cm/m2/3 had a volume of less than 6 ml/m2; both required shunt procedures. It therefore appears that if either the ventricular volume or tricuspid anulus size is excessively small, a shunt procedure is necessary. Wall thickness, area change fraction and ejection fraction measurements were not significantly correlated with right ventricular volume or postoperative outcome.(ABSTRACT TRUNCATED AT 250 WORDS)     

Extensive myocardial fiber disarray in aortic and pulmonary atresia. Relevance to hypertrophic cardiomyopathy

Myocardial fiber disarray is a distinctive histopathologic finding seen in asymmetric hypertrophic cardiomyopathy. We studied 14 hearts with aortic atresia and intact interventricular septum, six hearts with pulmonic atresia and intact interventricular septum, eight normal infant hearts matched for age of the study hearts, and one nonadult heart with hypertrophic cardiomyopathy and asymmetric hypertrophy and quantitatively analyzed tissue sections through both ventricles and the septum. Normal hearts had an average overall fiber disarray of 8.7% (range 3.8-17%) of the left ventricle including septum. Hearts with pulmonary atresia had an overall disarray of 70.4% (range 13-97%) of the entire right ventricle, and those with aortic atresia 62.1% (range 26-97%) of the left ventricle. The one infant heart with hypertrophic cardiomyopathy showed 15.5% disorder of the left ventricular free wall, 75% disorder of the septum and 47% overall myocardial fiber disarray. Thus, while quantitative criterion distinguished normal from abnormal hearts, they did not distinguish among the various pathologic states. Although extensive myocardial fiber disarray is not exclusive to, or pathognomonic of, hypertrophic cardiomyopathy, it is a useful finding taken in the context of the overall disease. The sensitivity and specificity of this isolated morphologic observation as an indication of hypertrophic cardiomyopathy may be misleading.