Transcatheter Closure of Right‐to‐Left Interatrial Shunts to Resolve Hypoxemia

Background. Right‐to‐left interatrial shunts through an atrial septal defect or patent foramen ovale may result from congenital and acquired conditions. Right‐to‐left shunts may occur with normal pulmonary artery pressure and resistance as in right ventricular outflow tract obstruction leading to hypertrophy and reduced diastolic function, in right ventricle ischemia, myocarditis or cardiomyopathy and in orthodeoxia–platypnea syndrome. Methods. We have tested the tolerance of trans‐catheter closure of right‐to‐left interatrial shunts in 5 selected patients in whom the morphology and hemodynamics implied that it would be well‐tolerated and improve functional capacity. Right‐to‐left shunt was demonstrated in 5 profoundly cyanotic patients by trans‐thoracic and trans‐esophageal echocardiography with intravenous injection of agitated saline. The patients were catheterized for hemo‐dynamic study and tested for tolerance of transient balloon occlusion of the defects. Results. Diastolic right ventricular dysfunction with elevated end‐diastolic pressure was the primary cause of right‐to‐left shunt. Most shunts occur via atrial septal defects. Patients' ability to tolerate temporary balloon occlusion of the defects predicted a favorable outcome following permanent device occlusion. Cyanosis resolved in all patients following closure of the defects without congestive right heart failure. A marked improvement in functional capacity was observed in 4 patients. One died of preinterventional hypoxic brain damage. Conclusion. Transcatheter closure of right‐to‐left shunts is well‐tolerated and a rewarding approach. It should be applied in selected patients following careful morphologic and hemodynamic evaluation. Tolerance of temporary occlusion of the defect is predictive of a favorable procedural outcome.     

Isolated atrial inversion Visceral situs solitus, visceroatrial discordance, discordant ventricular d loop without transposition, dextrocardia: Diagnosis and surgical correction

After cardiac catheterization and angiocardiographic studies an infant with cyanosis from birth was found to have visceral situs solitus, visceroatrial discordance without venoatrial discordance, a discordant ventricular d loop without transposition, and dextrocardia. The circulatory pathway was from the venae cavae to right atrium to left ventricle to aorta, and from the pulmonary veins to left atrium to right ventricle to pulmonary artery. An atrial defect was present allowing some mixing between the pulmonary and systemic circulations, but this appeared inadequate and the defect was enlarged surgically. Subsequently, a corrective operation using an atrial baffle to direct the pulmonary venous return to the left ventricle and the systemic venous return to the right ventricle was successfully undertaken.     

Double outlet right ventricle with severe left ventricular outflow tract obstruction due to small ventricular septal defect and anomalous adherence of the mitral valve to the ventricular septum

A 1-month-old male infant with a double outlet right ventricle complicated by left ventricular outflow tract obstruction due to a very small ventricular septal defect is described. The atrial septum was intact, but mitral stenosis was present. The characteristic finding in this patient was that the inherently small ventricular septal defect was further narrowed by abnormal adherence of the anterior mitral leaflet to the left ventricular septal surface below the defect. Intracardiac anatomical features other than the adherent mitral valve could be evaluated accurately by two-dimensional echocardiography. Because the atrial septum was intact, balloon atrioseptostomy was not possible. An attempt at surgical creation of an atrial septal defect was unsuccessful, and the patient died immediately after the operation. The autopsy findings of the heart are described, and diagnostic problems and the possibility of radical operation are discussed.     

组织多普勒成像在房间隔缺损封堵术中的应用

目的:利用组织多普勒成像（DTI）观测房间隔缺损封堵术前、后心脏功能的变化,以评价疗效和术后恢复情况。方法:房间隔缺损封堵术患者100例,分别于术前、术后3d、3个月和6个月进行超声心动图检查,于心尖四腔切面分别取右室侧壁三尖瓣环处、右室侧壁中部、室间隔二尖瓣环处、室间隔中部、左室侧壁二尖瓣环处以及左室侧壁中部组织多普勒速度频谱,测量收缩期（S）、舒张早期（E）和舒张晚期（A）峰值速度,计算E/A比值。结果:术前右室侧壁三尖瓣环处和右室侧壁中部各参数较大,室间隔侧二尖瓣环处和室间隔中部各参数较小;术后3d右室各运动参数显著降低,室间隔和左室S和E峰显著降低,但A峰参数无统计学变化;术后3个月右室、室间隔侧各运动参数均较术前显著降低,左室侧S峰较术前降低,余参数无统计学变化;术后6个月右室侧、室间隔二尖瓣环处各运动参数均较术前显著降低,但较术后3个月有增高趋势,室间隔中部各参数较术前无显著变化,左室S峰较术前显著降低。在各时间点中所有取样点的E/A比值无显著变化。结论:DTI能准确、定量观测房间隔缺损封堵术前、后心脏功能的变化,评价封堵术的治疗效果和恢复情况。     

First Report of Atretic Coronary Sinus Stenting in a 5‐kg Infant Resulting in Dramatic Improvement of Ventricular Function in Functional Single Ventricle

The patient was a male infant with L‐transposition of great arteries (L‐TGA), Ebstein's anomaly of the tricuspid valve, subvalvar aortic stenosis, ventricular septal defect (VSD), hypoplastic right ventricle, arch hypoplasia, and congenital complete heart block. He underwent a Norwood procedure, aortic arch repair, permanent pacemaker implantation, and a 3.5‐mm aortopulmonary shunt at 4 days of age. At the time of his surgery, left ventricular function was in the normal range (ejection fraction [EF] = 67%). However by 3 months of age, he was noted to have developed moderate–severe biventricular dysfunction (left ventricular ejection fraction [LVEF] = 34%). Atresia of the coronary sinus with a small left superior venacava (LSVC) and a bridging vein was discovered during cardiac catheterization at this time. The coronary sinus mean pressure was 17 mm Hg, and the common atrial mean pressure was 6 mmHg. We opened the atretic coronary sinus ostium using radiofrequency ablation and stent placement. There was dramatic improvement in ventricular function observed over a 2‐month period. Follow‐up cardiac catheterization 5 months later revealed the stent in the coronary sinus to be widely patent with no intimal buildup, and the ventricular function was normal (LVEF =58%). The patient had a bidirectional Glenn procedure with an uncomplicated postoperative course and is currently awaiting Fontan completion.     

Total correction of double-outlet right ventricle, atrial septal defect, pulmonic stenosis with a concordant atrioventricular relationship, dextrocardia and situs inversus

Surgical correction of a rare type of double-outlet right ventricle was successfully carried out in a 20-year-old man. The malformation was associated with atrial septal defect, ventricular septal defect, pulmonic stenosis, a concordant atrioventricular relationship, and dextrocardia with situs inversus. A Teflon patch was sutured as a baffle in the right ventricle to direct blood that had passed from the left ventricle through the ventricular septal defect out through the aorta. The infundibular obstruction was resected, a pulmonic commissurotomy performed and the atrial septal defect closed. The patient made an uneventful recovery and was well at follow-up 1 year later.     

Hypoplasia of the right ventricle with inter-atrial communication and without any other abnormality. Apropos of a case treated surgically by closure of the inter-atrial communication]

A case is reported of hypoplasie of the right ventricle with a wide atrial septal defect in a child of 10. The presence of cyanosis with right atrial and left ventricular preponderance led us to suspect tricuspid atresia, but this was excluded by catheterisation and angiocardiography. There was definitely and atrial septal defect with a two-directional shunt, but the tricuspid valve could be negotiated, and the right-sided opacity provided the essential diagnostic criterion, namely hypoplasia of the sinus portion of the right ventricle with a normal infundibulum. 17 other published cases of hypoplasia of the right ventricle with atrial septal defect are reviewed. The current indications for surgery are discussed in the light of the nature of the disordered physiology. Simple closure of the atrial septal defect, which was carried out successfully in 3 cases, appears to be of great value in the larger child, when there is a wide defect.     

Parachute Tricuspid Valve in a Patient with Atrial Septal Defect Detected by Two‐ and Three‐Dimensional Echocardiography

Atrioventricular valve parachute deformity was generally seen in the mitral position, which is called parachute mitral valve. Parachute deformity is also seen in the tricuspid position, but this has rarely been reported as sporadic cases. All of these cases were associated with other congenital defects, such as double outlet right ventricle, left to right shunt. We herein present two‐ and three‐dimensional echocardiographic features of a parachute tricuspid valve in a patient with atrial septal defect and prominent eustachian valve.     

Unexplained Double‐Chambered Left Ventricle Associated with Contracting Right Ventricular Aneurysm and Right Atrial Enlargement

In this article, we describe a double‐chambered left ventricle (LV) associated with a functional right ventricular (RV) aneurysm and right atrial (RA) enlargement in an asymptomatic 24‐year‐old woman with a family history of sudden cardiac death. We will discuss the differential diagnosis, genetic testing and possible prognostic implications.     

Comparative response of right and left ventricles to volume overload.

The cardiac volume data of 49 normal children were compared with those of 23 with secundum atrial septal defect and 24 with patent ductus arteriosus. Significantly smaller ventricular end-diastolic volumes were observed in the normal infants than in older children (right ventricle 53.9 versus 75.5 cm3/m2; left ventricle 46.7 versus 63.6 cm3/m2). "Distensibility" of the right ventricle (DRV), left ventricle (DLV) and left atrium increased normally with age. DRV and DLV were similar shortly after birth; thereafter, DRV increased more rapidly than DLV (mean DRV 12.7; mean DLV 7.8 cm3/m2 per mm Hg, P less than 0.001). In both atrial septal defect and patent ductus arteriosus, the ipsilateral (involved) ventricles had increased volume, increased output, normal ejection fraction and increased distensibility. The contralateral (left) ventricle in atrial septal defect was smaller than normal (39.6 versus 49.7 cm3, P less than 0.001), and had a smaller ejection fraction (0.63 versus 0.71, P less than 0.01) and output (3.70 versus 4.57 liters/min per m2, P less than 0.005). In contrast, the contralateral (right) ventricle in patent ductus arteriosus remained normal. Left atrial maximal volume was larger than normal in atrial septal defect (46.6 versus 35.9 cm3/m2, P less than 0.001). The left atrial and left ventricular volumes in patent ductus arteriosus were, respectively, 152 and 142 percent of normal, indicating comparable response to the volume load. The left head changes in atrial septal defect may be related both to a functionally restrictive defect and to the difference in distensibility of the ventricles.     

Three‐Dimensional Transthoracic Echocardiography in Identification of Aorto‐Right Atrial Fistula and Aorto‐Right Ventricular Fistulas

We report the case of a 72‐year‐old woman who developed new onset right ventricular failure after redo aortic valve replacement. The diagnosis of left to right shunt was initially made using two‐dimensional transthoracic echocardiography (2DTTE) and 2D transesophageal echo with color Doppler (TEE). Definite diagnosis of aorto‐right atrial and aorto‐right ventricular fistula was made using thee‐dimensional transthoracic echocardiography (3DTTE) with color flow Doppler imaging. Early recognition and diagnosis of this rare surgical complication is imperative for prompt surgical repair of this lethal defect. 3DTTE should be utilized in cases of new onset heart failure with unclear etiology to diagnose unusual causes of this potentially fatal condition. (Echocardiography 2010;27:E105‐E108)     

A novel method of hybrid intraoperative catheter‐based closure of ventricular septal defects using the Amplatzer® PDA occluder

Background: In five patients, an apical muscular septal defect was closed in a hybrid approach using the Amplatzer® duct occluder during open heart surgery, whereas concomitant defects were treated surgically. In addition to their different heart defects that needed surgery, all had a muscular ventricular septal defect in the apex of the heart, poorly accessible for traditional, surgical approach. We describe the method and outcome in these patients. Methods: The tip of a forceps was advanced from the left into the right ventricle through the ventricular septal defect. The delivery sheath was caught under visual control in the right ventricle and pulled back into the left ventricle. The disc was developed and pulled back until it was felt tugging at the septum. Then the core was developed. The end of the device was visible in the right ventricle and was secured with a Prolene® suture. Results: The procedures were event‐free. During early follow‐up there were either no or only insignificant shunts in the region of the prior trabecular defects in four patients. The unsatisfactory result in the last patient was caused by inaccurate preoperative assessment. Conclusion: The method seems valuable in patients in need of other intracardiac surgery. © 2010 Wiley‐Liss, Inc.     

Absent right atrioventricular connexion with a straddling left atrioventricular valve connecting the left atrium to a ventricular mass with a dominant left ventricle

We present clinical and postmortem examination of a child who presented in life with severe obstruction to the left ventricular outflow tract. The heart was right-sided with usual atrial arrangement. The right atrioventricular connexion was absent. There was straddling of the left atrioventricular valve which had a double orifice. Each orifice was connected to a well-differentiated ventricle, the left ventricle being dominant and right-sided (left-hand topology). An inlet ventricular septal defect was restricted by the leaflet tissue of the straddling valve.     

Cryoablation and Angiographic Evidence of a Concealed Right Atrial Appendage to Right Ventricle Accessory Pathway in an Infant

We report a case of successful cryoablation of a concealed accessory pathway from the right atrial appendage to the right ventricle in an infant. A 4‐month‐old female who had previously undergone successful ablation of a left‐sided accessory pathway was taken to the electrophysiology lab due to recurrent supraventricular tachycardia. While in this second supraventricular tachycardia, we found earliest atrial activation in the distal right atrial appendage. Angiography confirmed a connection between right atrial appendage and the right ventricle. Cryoablation at this location promptly terminated the supraventricular tachycardia. Following ablation, there were no further inducible arrhythmias and ventriculo‐atrial dissociation was appreciated.     

Atrioventricular discordance without ventriculo-arterial discordance. Apropos of 3 cases

Three cases of atrioventricular without ventriculo-arterial discordance are reported. The first case was a young woman with situs solitus, atrioventricular discordance and ventriculo-arterial concordance, large atrial and small ventricular septal defects. The functional tolerance was relatively good. The second case was an infant girl with dextrocardia by dextroversion and abdominal situs solitus. The child had atrioventricular discordance with ventriculo-arterial concordance and an ostium primum atrial septal defect. Cyanosis was mild and, in addition, complete heart block was diagnosed on the fourth day of life. The functional tolerance was good in this case too and growth was normal. The third case was a young girl with levocardia, atrial situs inversus, and abdominal situs inversus. A double outlet right ventricle was diagnosed at catheterisation with atrioventricular discordance, ventricular septal defect, and severe valvular and infundibular pulmonary stenosis. The first palliative procedure was performed at eight months: a left Blalock-Taussig anastomosis. As cyanosis has recurred a second procedure is being considered, the child now being 5 years old. Different forms of atrioventricular discordance without ventriculo-arterial discordance have been described: atrioventricular discordance with ventriculo-arterial concordance; atrioventricular discordance with double outlet right ventricle; atrioventricular discordance with double outlet left ventricle; atrioventricular discordance with a single vessel issuing from the right or left ventricle. With reference to their personal cases and those described in the literature, the authors describe the anatomy, physiopathology and the clinical consequences of the different forms encountered in their cases. The different surgical techniques for each anatomical form of this condition are discussed.     

Concomitant Gerbode‐Like Defect and Anterior Mitral Leaflet Perforation after Aortic Valve Replacement for Endocarditis

We present a case of concomitant left ventricle (LV) to right atrial shunt (Gerbode‐like defect) and anterior mitral leaflet perforation in a 32‐year‐old male after aortic valve replacement for infective endocarditis of bicuspid aortic valve. This case emphasises that intra‐operative transesophageal echocardiography is a sine qua non for valvular surgical procedures.     

Successful treatment of right ventricular failure with atrial septostomy

In the case reported, a patient with severe right ventricular failure following coronary revascularization was successfully weaned from cardiopulmonary bypass following creation of an atrial septal defect. This technique facilitated rapid decompression of the failing right ventricle by shunting blood to the more compliant left ventricle, thus augmenting left ventricular preload and enhancing cardiac output. Recovery of right ventricular function was demonstrated by progressive hemodynamic improvement, as well as reduction of right-to-left intracardiac shunting and resolution of arterial hypoxemia.     

Percutaneous closure of an atrial septal defect in an infant with shone's syndrome

Rarely is closure of the atrial septal defect indicated in infants. Yet, an atrial septal defect in the presence of congenital mitral stenosis may cause significant congestive heart failure in infancy. We present the case of an infant with Shone's syndrome, left ventricular hypoplasia, and a large ostium secundum atrial septal defect who developed pulmonary overcirculation following repair of coarctation of the aorta. The infant underwent cardiac catheterization, hemodynamic assessment, and successful percutaneous closure of the atrial septal defect. The patient improved dramatically following the intervention. Follow‐up echocardiograms have demonstrated growth of mitral valve annulus diameter and left ventricular dimensions. Atrial septal defect closure in this small infant was associated with immediate improved clinical status, and improved growth of the left heart structures. © 2012 Wiley Periodicals, Inc.     

Successful extensive enlargement of a non-committed ventricular septal defect in double outlet right ventricle

We performed an arterial switch operation in a patient with double outlet right ventricle with non-committed ventricular septal defect, and abnormal insertion of the tension apparatus of the tricuspid valve which produced moderate tricuspid regurgitation. This required extensive enlargement of the ventricular septal defect between the attachments of the cords of the tricuspid valve so as to create the interventricular rerouting that made possible the arterial switch operation. Postoperatively, we produced a straight, unobstructed, left ventricular outflow tract, improved the extent of tricuspid regurgitation, and achieved low right atrial pressures. Enlargement of the interventricular communication can set the scene for biventricular repair in this particular subset of patients with both arterial trunks arising from the morphologically right ventricle.     

An unusual tethering of the bridging leaflets in atrioventricular septal defect producing a communication from left atrium to right ventricle.

We describe a 39-year-old woman who was diagnosed as having an unusual atrioventricular septal defect with a communication from left atrium to right ventricle. A common atrioventricular junction, with partially separated right and left atrioventricular orifices, was found at transoesophageal ultrasonic examination. Both bridging leaflets were attached to the underside of the atrial septum, which was grossly malaligned relative to the ventricular septum. The shunt was exclusively from left atrium to right ventricle because of the overriding of the left atrioventricular valve, with the left component of the inferior bridging leaflet firmly fused to the ventricular septal crest.