Double outlet right ventricle with atrioventricular discordance. Report on cases having situs solitus, dextrocardia, pulmonary stenosis and levomalposition of the aorta

We describe six patients with situs solitus of viscera and atria, dextrocardia, atrioventricular discordance, ventricular septal defect, double outlet from the morphological right ventricle, pulmonic stenosis and levomalposition of the aorta. Four patients were male and two female; their age ranged from 3.5 to 31 years (mean 13.8 years). All had various degrees of disability, and presented with cyanosis, clubbing and high hematocrit levels. One patient had an atrio-ventricular block that varied from first to third degree; another patient showed intermittent junctional rhythm. At angiography the ventricular septum appeared to be almost perpendicular to the frontal plane in most cases, so that the anteroposterior projection resulted in a true axial view. One overriding left atrioventricular valve and one straddling right atrio-ventricular valve were demonstrated; no significant incompetence of either valve was observed. The ventricular septal defect was always single and related to the subpulmonary outflow. Pulmonic stenosis was valvular in every patient; an additional infundibular obstruction was present in one case. In two cases an additional stenosis was discovered at the supravalvular level. The left pulmonary branch was stenotic in one case; it was hypoplastic, with controlateral dilatation, in two cases; both pulmonary arteries were dilated in one case. The aorta was always to the left of the pulmonary artery, either anterior or side by side. Three patients were operated on in different Institutions: one had a pulmonic valvotomy at the age of six years; one, aged twenty, had a right Blalock-Taussig shunt; the third, with overriding left atrioventricular valve, underwent a modified Fontan operation at the age of thirty years with success.(ABSTRACT TRUNCATED AT 250 WORDS)     

Straddling mitral valve with hypoplastic right ventricle, crisscross atrioventricular relations, double outlet right ventricle and dextrocardia: morphologic, diagnostic and surgical considerations

The clinical, surgical and morphologic findings in five cases of a rare form of straddling mitral valve are presented. Three patients were diagnosed by two-dimensional echocardiography, cardiac catheterization and angiocardiography and two had diagnostic confirmation at autopsy. All five cases shared a distinctive and consistent combination of anomalies: 1) dextrocardia; 2) visceroatrial situs solitus, concordant ventricular D-loop and double outlet right ventricle with the aorta positioned to the left of and anterior to the pulmonary artery; 3) hypoplasia of right ventricular inflow (sinus) with tricuspid valve stenosis or hypoplasia; 4) large right ventricular infundibulum (outflow); 5) malalignment conoventricular septal defect; 6) straddling mitral valve with chordal attachments to the left ventricle and right ventricular infundibulum; 7) severe subpulmonary stenosis with well developed pulmonary arteries; and 8) superoinferior ventricles with crisscross atrioventricular (AV) relations. The degree of malalignment between the atrial and ventricular septa was studied quantitatively by measuring the AV septal angle projected on the frontal plane. The AV septal angle in the two postmortem cases was 150 degrees, reflecting marked malalignment of the ventricles relative to the atria. This AV malalignment appears to play an important role in the morphogenesis of straddling mitral valve. As judged by a companion study of seven postmortem cases, the more common form of straddling mitral valve with a hypertrophied and enlarged right ventricular sinus had less severe ventricular malposition than did the five rare study cases with hypoplastic right ventricular sinus. A competent mitral valve, low pulmonary vascular resistance and low left ventricular end-diastolic pressure were found at cardiac catheterization in the three living patients who underwent a modified Fontan procedure and are doing well 2.2 to 5.8 years postoperatively.     

Abnormal cardiac signs after Fontan type of operation: indicators of residua and sequelae.

Among 74 survivors of the Fontan type of operation abnormal cardiac signs were detected in 46 (62%) at postoperative examination. The findings were analysed in relation to the state of the cardiovascular system of these patients. Cyanosis was present in 10 (13.5%) patients. The causes of cyanosis included residual interatrial shunt (six patients), acquired pulmonary arteriovenous fistulas (three patients) and acquired systemic-to-pulmonary vein communication (one patient). Signs of chronic fluid retention were detected in six (8%) patients. In four of them the fluid retention was related to conduit obstruction and in the remaining two it was secondary to severe subaortic stenosis in one and atrioventricular valvar regurgitation in the other. Organic heart murmurs were heard in 29 (39%) patients. The aetiologies of these murmurs were multiple. They included aortic valve regurgitation (eight patients), subaortic stenosis (seven patients), atrioventricular valvar regurgitation (five patients), pulmonary valve regurgitation (five patients), residual Blalock-Taussig shunt (two patients), residual ventricular septal defect (two patients), residual communication in the main pulmonary artery which had been ligated but not divided (one patient), and left ventricular to right atrial shunting (one patient). Cardiac rhythm disturbances of varying aetiology were noted in 23 (31.1%) patients. Sixteen (21%) had supraventricular arrhythmias and seven (9.5%) had conduction abnormalities. The present review suggests that among survivors of the Fontan type of operation abnormal cardiac signs are indicators of residua or sequelae or both of the native cardiovascular anomalies or surgical procedures.     

Abnormal cardiac signs after Fontan type of operation: indicators of residua and sequelae

Among 74 survivors of the Fontan type of operation abnormal cardiac signs were detected in 46 (62%) at postoperative examination. The findings were analysed in relation to the state of the cardiovascular system of these patients. Cyanosis was present in 10 (13.5%) patients. The causes of cyanosis included residual interatrial shunt (six patients), acquired pulmonary arteriovenous fistulas (three patients) and acquired systemic-to-pulmonary vein communication (one patient). Signs of chronic fluid retention were detected in six (8%) patients. In four of them the fluid retention was related to conduit obstruction and in the remaining two it was secondary to severe subaortic stenosis in one and atrioventricular valvar regurgitation in the other. Organic heart murmurs were heard in 29 (39%) patients. The aetiologies of these murmurs were multiple. They included aortic valve regurgitation (eight patients), subaortic stenosis (seven patients), atrioventricular valvar regurgitation (five patients), pulmonary valve regurgitation (five patients), residual Blalock-Taussig shunt (two patients), residual ventricular septal defect (two patients), residual communication in the main pulmonary artery which had been ligated but not divided (one patient), and left ventricular to right atrial shunting (one patient). Cardiac rhythm disturbances of varying aetiology were noted in 23 (31.1%) patients. Sixteen (21%) had supraventricular arrhythmias and seven (9.5%) had conduction abnormalities. The present review suggests that among survivors of the Fontan type of operation abnormal cardiac signs are indicators of residua or sequelae or both of the native cardiovascular anomalies or surgical procedures.     

Hybrid trans‐apical atrioventricular valve‐in‐valve implantation in Fontan circulation

Trans‐apical approach has been proved successful in failing surgical bio‐prosthesis in both mitral and aortic position in adult patients. Recently, valve‐in‐valve treatments have been applied even in patients with complex congenital heart disease. Here, we report the case of a 32 years old lady with left atrial isomerism, complete AV septal defect, interrupted inferior vena cava with azygos continuation who underwent Kawashima procedure with atrial Fontan. Severe systemic atrioventricular valve regurgitation necessitated a 33 mm Perimount valve implantation and conversion to lateral tunnel Fontan. After only 4 years there was severe valve stenosis and the patient underwent successful trans‐apical transcatheter implantation of a 29 mm Sapien valve.     

Double-outlet left ventricle: Diagnosis and management

Double-outlet left ventricle (DOLV) is a rare congenital cardiac malformation in which both great arteries originate entirely or predominantly from the morphologic left ventricle. DOLV occurs most commonly in the form of atrial situs solitus with atrioventricular (AV) concordance. Ventricular septal defect (VSD) is most commonly subaortic, and 90% of these patients have associated pulmonary stenosis. In most cases, there is a right anterior position of the aorta relative to the pulmonary artery. Most patients with DOLV will have pulmonary outflow obstruction and present soon after birth with cyanosis. DOLV with two well-developed ventricles is conventionally treated surgically by VSD closure, closure of the native left ventricle-to-pulmonary outflow, and placement of a right ventricle-to-pulmonary artery extracardiac conduit. However, pulmonary root translocation may be used as an alternative to biventricular repair of DOLV. Patients with DOLV and functionally single-ventricle or AV valve atresia will require a Fontan procedure.     

Accessory valve cusp as a cause of outflow tract obstruction in atrio-ventricular and ventriculo-arterial discordance

Congenital corrected transposition of the great arteries in which there is both an atrio-ventricular and a ventriculo-arterial discordance has surgical significance only in consequence of the associated cardiac anomalies. Their surgical correction is subject to multifarious problems. An exceedingly rare cause of a left-ventricular outflow tract obstruction erroneously interpreted preoperatively in an eight-year-old boy is reported. In an l-transposition with inversion of both ventricles, the diagnosis "valvular pulmonary stenosis" was made preoperatively. However, a normally arranged tricuspid pulmonary valve without stenosis was revealed intraoperatively. On the other hand, dystopic valvular tissue in the form of a monocuspid atrioventricular valve which was attached to an accessory papillary muscle with several tiny tendons was found below the pulmonary valve in the morphological left ventricle. The accessory valve cusp, which was the cause of the hemodynamically relevant subvalvular pulmonary stenosis, had no connection with the mitral valve. The abnormal valve cusp was resected without injuring the normal structures. The angiographic follow-up after one year no longer revealed any pressure gradient. This case report points out the possibility of an outflow tract obstruction caused by a complete accesory valve cusp.     

Staged septation of double inlet left ventricle

Complete septation of a double inlet left ventricle with left anterior subaortic outlet chamber was carried out successfully as a staged procedure during the first two years of life in a patient with severe pulmonary hypertension and an abnormal left atrioventricular valve. In contrast with isolated pulmonary artery banding, which rarely has led to a satisfactory septation or modified Fontan operation, this technique achieved good initial palliation and uncomplicated intracardiac repair. It should be considered for infants with univentricular atrioventricular connection, two atrioventricular valves, and excessive pulmonary blood flow.     

Staged septation of double inlet left ventricle.

Complete septation of a double inlet left ventricle with left anterior subaortic outlet chamber was carried out successfully as a staged procedure during the first two years of life in a patient with severe pulmonary hypertension and an abnormal left atrioventricular valve. In contrast with isolated pulmonary artery banding, which rarely has led to a satisfactory septation or modified Fontan operation, this technique achieved good initial palliation and uncomplicated intracardiac repair. It should be considered for infants with univentricular atrioventricular connection, two atrioventricular valves, and excessive pulmonary blood flow.     

Spontaneous Obstruction of Interatrial Communication in Infants with Atrioventricular Valve Atresia or Stenosis in the Neonatal Period: Serial Echocardiographic-Doppler Study

BACKGROUND AND METHODS: The objective of this serial echocardiographic study was to document the critical size of the interatrial communication in infants with atrioventricular valve atresia, with which spontaneous obstruction is most likely to occur. Spontaneous obstruction of interatrial communication (patent foramen ovale) has been reported to occur in infants with atrioventricular valve atresia or severe stenosis. Atrial septostomy or septectomy therefore may be considered during initial palliation of these infants. Serial two-dimensional Doppler echocardiographic examinations were performed on 11 newborn infants with complex cyanotic congenital cardiac lesions for whom Fontan procedure was contemplated. These newborn infants had either mitral or tricuspid valve atresia or severe stenosis other than hypoplastic left heart syndrome. The size of the foramen ovale was measured from the subcostal frontal, long-axis oblique, and parasagittal views. Conventional Doppler and color flow mapping of the interatrial shunt also was obtained on each patient. RESULTS: The size of the foramen ovale was 5 mm in nine patients at birth. Five of these patients underwent Blalock-Taussig shunt in the neonatal period for severely restricted pulmonary blood flow. In three patients, surgery was postponed due to severe systemic (noncardiac) disease. One patient underwent the hemi-Fontan procedure without a previous shunt. In all nine patients, the foramen ovale remained the same size or became progressively smaller beyond the neonatal period, and atrial septectomy was performed in five patients after initial palliation. In the remaining two patients (who underwent Blalock-Taussig shunt as initial palliation), the foramen ovale measured >/=10 mm at birth and became progressively larger on subsequent follow-up studies. Atrial septectomy was not necessary in these patients. In the first nine patients, Doppler technique did not detect any obstructive pattern during the first 2-4 weeks of life, after which it became progressively obstructive (i.e., >2 m/sec and nonphasic). CONCLUSIONS: In newborn infants with either atrioventricular valve atresia or severe stenosis, the interatrial communication, which may not appear restrictive by Doppler at birth, becomes progressively obstructed beyond the neonatal period. If the size of the communication is <10 mm in diameter, as measured by two-dimensional echocardiography, atrial septectomy should be performed as an integral part of any initial palliation to protect the pulmonary vascular bed for subsequent successful Fontan repair.     

Infectious Brucella endocarditis in a case of corrected transposition of the great arteries

We describe a case of a 14 year old male who developed infections endocarditis due to Brucella sp. He had a corrected transposition of great vessels with valvular pulmonary stenosis and mitral insufficiency. Vegetations were demonstrated by echocardiogram on the pulmonary valve as well as on left atrioventricular valve. He had also proliferative nephritis. Prolonged antimicrobial treatment results in complete healing. Because the rarity of this association we inform this observation.     

Is there another type of biventricular atrioventricular connection?

In evaluating a 9-year-old girl, we encountered the following cardiac anomalies: a left atrioventricular valve (the morphologic left atrium on the left side, connected with the morphologic left ventricle); concordant atrioventricular connections; a right atrioventricular valve (a morphologic right atrium on the right side, connected with the morphologic right ventricle); concordant atrioventricular connection; a "central" atrioventricular valve (separating the morphologic right atrium from the morphologic left ventricle); and discordant atrioventricular connection with intact interatrial and interventricular septa. A right-to-left shunt passed through the central valvular structure, which was situated at the atrioventricular septum. This was the only means of mixing pulmonary and systemic blood. We suggest that this case presents a newly recognized cardiac anomaly.     

Percutaneous transcatheter occlusion of left atrioventricular valve in a patient with single ventricle and severe atrioventricular valve regurgitation

Many patients with single ventricle physiology suffer from atrioventricular valve (AVV) regurgitation which may worsen their cardiac function and cause symptoms. It has been postulated that elimination of the nondominant hypoplastic AVV regurgitation, might improve the clinical status in patients post-Fontan surgery. We describe a case of hypoplastic left heart variant, post Fontan surgery who had severe left AVV regurgitation and underwent percutaneous transcatheter occlusion of the hypoplastic left AVV, using a VSD occluder device. At 3 months post procedure, the patient is improved. Transcatheter closure of a regurgitant hypoplastic AVV in a patient with single ventricle helps to improve the patient's cardiac function and clinical status.     

The univentricular heart: combined 2-dimensional-pulsed Doppler (duplex) echocardiographic evaluation

Two-dimensional echocardiographic-pulsed Doppler (2D-PD) evaluation was performed in 14 patients with a univentricular heart. Accurate information on ventricular structure, atrioventricular valve status, and great vessel anatomy and function was obtained. The characteristic morphologic features were verified in each case by angiography or autopsy, or both. In several patients, 2D-PD evaluation provided more accurate anatomic and functional information than did cardiac angiography in standard views, particularly with regard to atrioventricular valve function. Four children with pulmonary atresia and 4 with pulmonary stenosis were identified prospectively. No patient had 2D-PD evidence of aortic obstruction. Four children had typical 2D-PD patterns consistent with atrioventricular valve regurgitation. Thus, 2D echocardiography coupled with a range-gated Doppler system will improve recognition of patients with a univentricular heart and permit thorough evaluation of specific anatomic details.     

New technique for fenestration of the interatrial septum

Achieving controlled flow between the systemic and pulmonary venous circulations is desirable in many complex congenital heart diseases. This includes the Fontan circulation, primary pulmonary hypertension, double inlet ventricles, or hypoplastic left heart with obstruction to the atrioventricular valve. As no specific device is available for this purpose, we developed a balloon-mounted stent technique to achieve a predetermined-sized fenestration of an atrial baffle in a patient with Fontan circulation. The details of the technique are described.     

Hypoplastic left heart syndrome: hemodynamic and angiographic assessment after initial reconstructive surgery and relevance to modified Fontan procedure

After undergoing initial reconstructive surgery for hypoplastic left heart syndrome performed between August 1985 and March 1989, 59 patients (age range 3 to 27 months, mean 13.8 +/- 4.5) underwent elective cardiac catheterization in anticipation of a modified Fontan procedure. Five important hemodynamic and anatomic features considered to be components of successful reconstructive surgery were specifically addressed. 1) Interatrial communication: Only two patients had a measured pressure difference of greater than 4 mm Hg across the atrial septum. 2) Tricuspid valve function: Angiography demonstrated significant tricuspid valve regurgitation in only five patients (moderate in two and severe in three). 3) Aortic arch: Pressure tracings from the right ventricle to the descending aorta revealed a gradient greater than 25 mm Hg in only two patients. 4) Pulmonary vasculature: Ten patients had a calculated pulmonary vascular resistance greater than 4 U.m2; 51 (86%) of the 59 patients had no evidence of distortion (stenosis or hypoplasia) of either the left or the right pulmonary artery. 5) Right ventricular function: Five patients had an end-diastolic pressure in the right ventricle greater than 12 mm Hg and two patients had qualitative assessment of decreased ventricular function. Comparison of catheterization data between survivors and nonsurvivors of the subsequent modified Fontan procedure showed that only significant tricuspid regurgitation is a possible predictor of poor outcome. After first stage reconstructive surgery for hypoplastic left heart syndrome, most survivors have favorable anatomy and hemodynamics at follow-up cardiac catheterization for a subsequent Fontan procedure.     

Partial and transient relief of conduit obstruction by low-pressure balloon dilation in patients with congenital heart disease

Seven patients underwent attempted low pressure balloon dilation of stenotic conduits or homografts from right ventricle to pulmonary artery (n=5), in the aortic valve position (n=1), or from right atrium to left pulmonary artery (n=1). In the right ventricle to pulmonary artery group, mean gradient reduction was only 17%. At follow-up, two patients underwent surgical conduit replacement, one had a stent implanted at cardiac catheterization, the other two are awaiting surgical intervention. The patient with a homograft in the aortic valve position had a good initial result but restenosed within 1 year and underwent a pulmonary autograft operation. The patient with the Fontan homograft stenosis had transient obstruction relief but subsequently required stent implantation. Low-pressure balloon dilation of conduits or homografts is only partially and transiently successful. Whether stent implantation will offer better long-term results remains to be determined.     

Direct atriopulmonary anastomosis (modified Fontan) for single ventricle and its equivalents

The modified Fontan procedure is being used in an increasing number of complex cyanotic cardiac lesions with pulmonary stenosis. Seven patients aged 11 to 24 years (average 17.5 years) underwent surgery by a technique derived from the Fontan procedure: direct atriopulmonary anastomosis without a tube or valve. The tricuspid valve when patent was closed with a patch. The diagnoses were: single ventricule (4 cases), Taussig-Bing anomaly (2 cases) and tricuspid atresia (1 case). All patients had associated pulmonary stenosis with low pulmonary vascular resistances. The great vessels were in L-malposition in 3 cases. The hospital mortality was nil. Transient atrial fibrillation was observed in 2 cases and was well tolerated clinically. The follow-up period ranges from 2 months to 4 years (average 2.3 years). All patients are acyanotic with no signs of right-sided failure and in sinus rhythm. Control cardiac catheterisation and angiography were performed in 6 cases and showed good function of the anastomosis and a mean atrial pressure of 14 mmHg. Direct atriopulmonary anastomosis offers a very acceptable surgical solution to certain forms of single ventricle or equivalent with low pulmonary pressures. The short and medium term results seem to be better than those of intraventricular repair.     

An unusual case of congenitally corrected transposition of the great arteries associated with noncompaction-like remodeling of the morphological right ventricle

Congenitally corrected transposition of the great arteries is a rare disease characterized by atrio-ventricular and ventriculo-arterial discordance (double discordance) and is associated with other cardiac anomalies in the majority of cases. The important associated anomalies include ventricular septal defect, abnormalities of left atrioventricular valve, subpulmonary stenosis, and conduction abnormalities. However, the noncompaction-like remodeling of the subaortic, morphologic right ventricle is not a commonly reported association. We, report a case of congenitally corrected transposition of the great arteries in a 40-year-old male patient, who had noncompaction-like remodeling of the morphologic right ventricle with severe ventricular dysfunction. He also had, left-sided Ebstein's anomaly, severe left atrioventricular valve regurgitation and a 2:1 atrioventricular conduction block.     

Cor Triatriatum Sinistrum Presenting as Cardioembolic Stroke: An Unusual Cause of Adolescent Hemiparesis

Cor triatriatum sinistrum is a rare congenital cardiac malformation, in which the left atrium (LA) is divided into two distinct chambers by a fibromuscular membrane. Classically, the proximal (upper or superior) chamber of the LA receives pulmonary venous connections, whereas the distal (lower or inferior) chamber contains LA appendage and true atrial septum containing fossa ovalis. The distal chamber is in continuity with the atrioventricular valve, while the two chambers communicate through a defect in the membrane. The hemodynamics of cor triatriatum sinistrum are similar to that of mitral stenosis due to obstructive property of membrane. The majority of reported cases of cor triatriatum occur in infants with symptoms of pulmonary venous obstruction, with adult cases being rare. Herein, we describe an unusual case of cor triatriatum in a 17‐year‐old boy who presented for the first time with embolic cerebral infarction with left hemiparesis.