Left-sided Atrioventricular Valve Research Articles

The Melody Valve in Small Children Undergoing First Mitral Valve Replacement: Better Than Mechanical?

BackgroundIn small children with left atrioventricular valve dysfunction, valve repair is preferred but some will require valve replacement. No prosthetic valve has growth potential, bioprostheses have poor durability, and mechanical prostheses have high rates of thromboembolic and hemorrhagic complications. We reviewed our experience with a modified bovine jugular vein valve designed for use in the right ventricular outflow tract (Melody valve, Medtronic) and compared this with contemporary mechanical valve replacement. MethodsWe reviewed outcomes of all left-sided atrioventricular valve replacements (AVVR) performed between January 2017 and April 2023 on patients with biventricular circulation less than 5 years old at time of surgery, N = 19. We compared mortality and major morbidity outcomes between mechanical AVVR (mAVVR, n = 14) and Melody AVVR (MAVVR, n=5). ResultsMAVVR was performed on younger and smaller patients (5.7 ± 4.2 vs 28.4 ± 22.3 months, P = .002; 6 ± 1.7 vs10 ± 5.1 kg, P = .01). There were 2 early and 1 late death in the mAVVR group, and none in the MAVVR group. There was a trend towards more hemorrhagic and neurologic complications in the mAVVR group (P = .1, 0.28, respectively). ConclusionsIn this small series, despite its use in younger and smaller patients, our preliminary experience with the Melody valve compares favorably with our contemporaneous outcomes with mechanical prostheses by way of simpler anticoagulation management with a trend towards better overall outcomes.

Redo mitral valve replacement in an adult with severe pulmonary hypertension resulting from structural valve deterioration and left ventricular outflow tract obstruction and a history of atrioventricular septal defect repair: a case report

BackgroundPulmonary hypertension (PH)-associated with left heart disease (Nice PH classification group II) improves when the latter is treated; however, the treatment of PH concomitant with group I PH due to congenital heart disease is difficult, and the optimal pharmacotherapy is controversial. Intervention strategies for the left-sided atrioventricular valve in partial atrioventricular septal defect (AVSD) are problematic.Case presentationA 37-year-old woman who had undergone patch closure for a partial AVSD and mitral valve replacement with a rather large bioprosthesis at the juxta-annular position for mitral regurgitation 12 years earlier was referred to our institute because of severe PH. Echocardiography revealed calcification resulting in severe stenosis of the bioprosthesis and protrusion of its stent post into the left ventricular outflow tract; therefore, redo mitral valve replacement at the supra-annular position was performed using a mechanical valve. Combined group I and II PH gradually improved with meticulous postoperative medical management.ConclusionsSevere PH due to stent post protrusion and structural valve deterioration in AVSD was successfully treated with redo mitral valve replacement. The present case was complicated with group I and II PH, for which medical therapy in conjunction with surgical treatment yielded an optimal therapeutic effect.

Corrected Transposition of the Great Arteries with Ebstein’s Anomaly, Dysplasia of the Mitral Leaflets and Persistence of Left Superior Vena Cava in an Adult

Introduction: Congenitally corrected transposition of the great arteries (cc-TGA) is a congenital heart disease rarely described among adult population, especially if it is associated with other abnormalities such as Ebstein’s anomaly and abnormal origin of coronary arteries. Case presentation: Twenty-two-year-old woman admitted to intensive care unit with acute decompensated heart failure. The transthoracic echocardiography demonstrated atrioventricular and ventriculoarterial discordance. The left-sided atrioventricular valve, in this case, the tricuspid valve, showed apical displacement of the septal valve suggesting Ebstein’s anomaly. The computed tomography corroborated these findings and additionally showed a left superior vena cava and an abnormal origin of the coronary arteries. The patient is in NYHA functional class II, receiving treatment with diuretic, digitalis, beta-blockers and angiotensin-converting enzyme (ACE) inhibitor. Conclusion: This is a very interesting case of an adult in the third decade of life with cc-TGA associated with abnormalities not described in the literature that had reached adulthood. Noninvasive images play an important role in its diagnosis.

Congenital heart disease in the older adult: a scientific statement from the American Heart Association.

The population of adults with congenital heart disease (ACHD) has increased dramatically over the past few decades, with many people who are now middle-aged and some in the geriatric age range. This improved longevity is leading to increased use of the medical system for both routine and episodic care, and caregivers need to be prepared to diagnose, follow up, and treat the older adult with congenital heart disease (CHD). The predictable natural progression of CHD entities and sequelae of previous interventions must now be treated in the setting of late complications, acquired cardiac disease, multiorgan effects of lifelong processes, and the unrelenting process of aging. Despite the advances in this field, death rates in the population from 20 to >70 years of age may be twice to 7 times higher for the ACHD population than for their peers.1 This American Heart Association (AHA) scientific statement will focus on the older adult (>40 years old) with CHD. It is meant to be complementary to the 2008 American College of Cardiology (ACC)/AHA guidelines for ACHD and orient the reader to the natural history, ramifications of childhood repair, and late initial diagnosis of CHD in the older adult. This population with CHD is unique and distinct from both the pediatric and young adult populations with CHD. Much of the information we provide is from scientific research combined with clinical experience from longitudinal care. We emphasize that this is the beginning of a discussion regarding this rapidly growing population, and continued research aimed at the progression of disease and complications reviewed here is necessary to advance the field of ACHD with the scientific rigor it deserves. ACHD encompass a broad range of presentations. There are people who are diagnosed for the first time in adulthood, as well as those with prior palliative repair …

Post-operative left atrioventricular valve function after the staged repair of complete atrioventricular septal defect with tetralogy of Fallot.

This study reviewed surgical outcomes of staged repair for complete atrioventricular septal defect with tetralogy of Fallot, especially with focusing on the post-operative left-sided atrioventricular valve function. Between 1992 and 2013, 10 patients with complete atrioventricular septal defect with tetralogy of Fallot underwent total correction by the following surgical strategy. Systemic-to-pulmonary shunt was placed at first at the mean age of 1.5 ± 1.3 months. Then confirming sufficient development of the left heart structures, the total correction was performed at the mean age of 1.4 ± 0.6 years. Second shunt was required in 4 (40 %) patients to develop the left heart structures. The left ventricular end-diastolic volume before total correction was 127 ± 30 % of normal size. The two-patch repair was applied in 8 (80 %) patients. There was no mortality, and 1 reoperation case for left-sided atrioventricular valve regurgitation. The follow-up was completed on all patients and the mean follow-up period was 7.4 ± 7.0 years. The post-operative left-sided atrioventricular valve regurgitation kept less than moderate for 10-year follow-up in all patients except one patient who required the repair of left-sided atrioventricular valve 1 year after the total correction. The post-operative left-sided atrioventricular valve function after the repair of complete atrioventricular septal defect with tetralogy of Fallot maintained with the application of the two-patch repair, early and repeated palliative systemic-to-pulmonary shunt, and the early definitive surgery.

The assessment of modified single-patch and two-patch techniques in complete atrioventricular septal defects and our short-term results

Background: This study aims to compare the postoperative shortterm results of the modified single-patch technique with those of the two-patch technique in infants with complete atrioventricular septal defects (AVSD). Methods: Twenty-nine patients ( 16 m ales, 13 females; mean age 7.7±7.8 months; range; 3-36 months) who were diagnosed with AVSD and underwent surgical repair between July 2007 and November 2009 in our clinic were retrospectively evaluated. The patients were divided into two groups depending on the surgical technique. In group 1, the modified single-patch technique (n=11; 38%) and in group 2, the modified two-patch technique (n=18; 62%) were used. Gender, body weight, body surface area, cross-clamp time, cardiopulmonary bypass time, duration of mechanical ventilation, preoperative and postoperative pulmonary arterial pressures, duration of intensive care unit stay, morbidity, mortality and postoperative echocardiographic results of the patients were compared in both groups. Results: Rastelli type A was seen in 10 patients (91%) in group 1 and in 16 patients (89%) in group 2 (p=0.86). Cardiopulmonary bypass and aortic clamp times were shorter in group 1 than in group 2 (96.7±25.1 vs. 138±63.9 minutes and 70.5±23.1 vs. 92.3±17.1, respectively; p=0.01). Postoperative mild left-sided atrioventricular valve insufficiency was observed in the echocardiographic evaluation of three patients (1 patient in group 1 and 2 patients in group 2). A moderate degree of left-sided atrioventricular valve insufficiency was observed only in one patient of group 1. Left ventricular outflow tract obstruction was not detected in either groups postoperatively. There were three (27%) deaths in group 1 and two deaths (11%) in group 2 in the early postoperative period. No mortality was observed during follow-up of 12 months after surgery. In group 2, one patient (5%) developed complete atrioventricular blockage in the postoperative period and a permanent pacemaker was implanted. Conclusion: The modified single-patch technique can be performed with acceptable results in infants with complete AVSD when compared with the double-patch technique. The modified single-patch technique can be performed with a shorter cardiopulmonary bypass time.

Percutaneous Transcatheter Closure of a Paravalvular Leak in a 20-Month Old Child

A 20-month-old boy was referred to the cardiac catheterization laboratory for closure of a large prosthetic mitral valve paravalvular leak. At birth, the boy had an unbalanced atrioventricular septal defect with borderline hypoplasia of the mitral component of the common atrioventricular valve and the left ventricle. At the age of 3 months, the boy underwent complete (two-ventricle) repair. The intraoperative findings included deficient left-sided atrioventricular valve tissue. As a result of the surgical septation, the coronary sinus ostium was located in the left atrium. The boy initially did well, but 6 weeks after his initial surgery, progressive and severe mitral regurgitation developed, necessitating mitral valve replacement with a 19-mm St. Jude prosthetic valve in the supraannular position. A small (posteromedial) paravalvular prosthetic mitral leak was noted approximately 6 months after surgery. This leak became progressively worse, resulting in moderate to severe functional mitral regurgitation and worsening left ventricular dilation. A dilated structure posterior to the left atrioventricular groove also was noted to be expanding. This structure was interpreted by echocardiography as an aneurysm of the coronary sinus. Three-dimensional (3D) echocardiography helped to define the anatomy of the leak (Fig. 1a/Movie 1a in supplementry material). At the age of 20 months, the boy was referred for cardiac catheterization with planned closure of the leak.

Outcome after reoperation for atrioventricular septal defect repair

Results of surgical repair of atrioventricular septal defect (AVSD), both partial (PAVSD) and complete (CAVSD), have improved. However, reoperation is not uncommon. This report describes our experience in 59 patients who underwent reoperation after AVSD repair, between 1977 and 2008. Thirty-one patients had a PAVSD, 28 had a CAVSD. Mean interval between initial repair and reoperation was 10+/-11 years (PAVSD vs. CAVSD: 13+/-12 vs. 6+/-9 years, P=0.063). Reoperations were required for left atrioventricular valve regurgitation (LAVVR) in 53 patients (combined with right atrioventricular valve regurgitation in 10, atrial septal defect (ASD) in 11, ventricular septal defect (VSD) in 7, left ventricular outflow tract (LVOT) obstruction in 1, and aortic valve stenosis in 1), ASD in 3, and LVOT obstruction in 3. Valve repair was performed in 45 patients and replacement in 8. Repair techniques of the left-sided atrioventricular valve (LAVV) included cleft closure in 44 patients, commissuroplasty in 19, and annuloplasty in 1. Freedom from additional reoperation was 85%, and 80% at 5 and 15 years. Hospital mortality was 3%. Overall survival was 91%, and 86% after 5 and 15 years. The most common indication to undergo reoperation is LAVVR. Reoperation is safe and in the majority of cases, a durable repair of the LAVV can still be achieved.

Atrioventricular septal defect with intact septal structures presenting as left atrioventricular valvar insufficiency

We describe the findings in a six-year-old girl who presented with signs of left atrioventricular valvar insufficiency. The echocardiogram showed a common atrioventricular junction, intact atrial and ventricular septal structures. At surgery, the left-sided atrioventricular valve was found to be tri-foliate, and corrected by valvoplasty. To the best of our knowledge, this is the first case of atrioventricular septal defect with common atrioventricular junction and intact septal structures diagnosed during life.

Predictive Value of Intraoperative Transesophageal Echocardiography in Complete Atrioventricular Septal Defect

Intraoperative transesophageal echocardiography and follow-up transthoracic echocardiography have been useful in assessing cardiac function in complete atrioventricular septal defects. However, it has been suggested that a discrepancy exists between intraoperative and postoperative findings, and that intraoperative findings cannot reliably predict long-term results. This study aims to determine whether this discrepancy exists and to assess whether it is possible to predict follow-up results using intraoperative transesophageal echocardiography. A retrospective analysis was made in 35 patients who underwent biventricular repair by one surgeon between November 1997 and January 2004. All patients received intraoperative transesophageal echocardiography and follow-up transthoracic echocardiography at 19.1 +/- 18.02 months (range, 7 days to 5 years; median, 15.1 months). In left-sided atrioventricular valve regurgitation, 34.3% (12 of 35) of patients showed discrepancy during follow-up, and 28.6% (10 of 35) showed progression of regurgitation (from grade I to II). In right-sided atrioventricular valve, 11.4% (4 of 35) of patients showed discrepancy, 9.6% (3 of 35) showed progression of regurgitation (from grade I to II). In complete atrioventricular septal defects, intraoperative transesophageal echocardiography did not show the same findings as that of follow-up transthoracic echocardiography in some cases. However, this discrepancy is not so great as to require reoperation in early to midterm follow-up. Therefore, intraoperative transesophageal echocardiography may be used as tool to predict durability of surgical results and to decrease the incidence of reoperation in complete atrioventricular septal defects.

Development of Left Atrioventricular Valve Regurgitation After Correction of Atrioventricular Septal Defect

Left-sided atrioventricular valve regurgitation is the main indication for reoperation in patients after repair of both partial and complete atrioventricular septal defect. Until now, the timing for reoperation is difficult. We sought to determine the outcome of severe residual left-sided atrioventricular valve regurgitation, either medically treated or reoperation. In this regard risk factors were determined for severe residual left-sided atrioventricular valve regurgitation and reoperation, and the most appropriate strategy for patients with postoperative severe left-sided atrioventricular valve regurgitation was identified. Retrospective review of clinical, operative, and echocardiographic data was performed. From 1990 until 2001 164 patients underwent correction of their atrioventricular septal defect. Five patients died in the immediate postoperative period, and 2 patients were lost to follow-up. During follow-up (median, 66 months; range, 9 months to 12 years), 30 patients (19%) had severe left-sided atrioventricular valve regurgitation. Sixteen patients had severe left-sided atrioventricular valve regurgitation in the immediate postoperative period; 4 of them showed spontaneous regression to near-normal valve function during follow-up. Fourteen patients exhibited left-sided atrioventricular valve regurgitation during follow-up; 8 of them remained stable with medication only. Fifteen of the 30 patients with severe left-sided atrioventricular valve regurgitation underwent reoperation. A significant risk factor for the development of severe left-sided atrioventricular valve regurgitation and reoperation was the presence of preoperative severe left-sided atrioventricular valve regurgitation. Severe left-sided atrioventricular valve regurgitation develops in a significant number of patients after correction of atrioventricular septal defect, and preoperative severe left-sided atrioventricular valve regurgitation is an important risk factor. Although reoperation usually results in good valve function, spontaneous regression after the immediate postoperative period is possible and should be given a fair chance.

Functional acquired pulmonary atresia secondary to subpulmonary aneurysmal tissue: a rare form of pulmonary outflow tract obstruction

artery (PA) arising from rudimentary pouch (*). Fetal echocardiography was performed at 19 and 29 weeks gestation after an abnormal screening ultrasound suggested left heart hypoplasia. The echocardiogram revealed left-sided hypoplasia with d-transposition of the great arteries and a small, posteriorly positioned pulmonary artery (PA) with both prograde and retrograde flow demonstrated. There appeared to be a moderate ventricular septal defect and, of note, there was unusual aneurysmal tissue of indeterminate origin below the pulmonary annulus. Postnatal echocardiography confirmed d-transposition of the great arteries with the aorta anterior and rightward of the smaller PA. The PA arose directly from a rudimentary, aneurysmal blind pouch (Figure 1) with bidirectional flow between the blind pouch and the main PA (Figure 2). However, no communication between the pouch and the remainder of the heart could be demonstrated by Doppler interrogation. The aneurysmal pouch was centrally located in the heart and appeared to possibly consist of redundant atrioventricular (AV) valve tissue contiguous with a leaflet of the left-sided AV valve. The PA was supplied by a large, tortuous patent ductus arteriosus typical for pulmonary atresia. There were 2 distinct AV valves entering a common single ventricle, with hypoplasia of the left-sided AV valve noted. The patient was initially treated with prostaglandin infusion to maintain ductal patency, followed by placement of a right-sided Blalock-Taussig shunt in the neonatal period. She subsequently underwent successful bidirectional Glenn anastomosis and left pulmonary arterioplasty at 7 months of age. This case represents an extremely unusual form of functional pulmonary atresia in which abnormal intracardiac aneurysmal tissue of indeterminate ori-

CONGENITALLY CORRECTED TRANSPOSITION OF THE GREAT ARTERIES WITH EBSTEIN MALFORMATION AND HYPOPLASIA OF THE AORTIC ARCH IN A FETUS

Congenitally corrected transposition of the great arteries is not uncommonly associated with Ebstein's malformation of its left-sided tricuspid valve. The recognition of this rare association in the fetus is important because its postnatal prognosis is guarded. The present case report illustrates fetal echocardiographic clues to the diagnosis that include mesocardia and apically displaced attachment of the left-sided atrioventricular valve to the ventricular septum at four-chamber view.

Surgical repair of double-orifice of the mitral valve in cases with an atrioventricular canal defects.

A Double-orifice in the mitral valve is an uncommon congenital cardiac lesion which occurs as an isolated anomaly or in association with other cardiac malformation. This report deals with our surgical experience of a double-orifice of the mitral valve in cases with an atrioventricular canal defect. From 1991 through 1999, ten patients were diagnosed to have a double-orifice of the mitral valve at Shizuoka Children's Hospital. Each patient had associated major cardiac malformations, among which atrioventricular canal defect underwent surgical management, with five of these undergoing complete correction with or without previous pulmonary artery banding. Of these 10, the five cases were enrolled in this study. Two of these had a complete type, and the other three had a partial type. The cleft in the left-sided atrioventricular valve was closed partially in four and left untouched in one. Bridging tissue, when present, was left intact. There was no regurgitation from any accessory orifice and no repair for an accessory orifice was needed. There was no late death and no replacement of the valve with prosthesis. During follow-up ranging from 1 to 4 years, none of the patients developed severe stenosis or progressive regurgitation in the left-sided atrioventricular valve. Meticulous surgical management of a double-orifice in the mitral valve in association with atrioventricular canal defect an achieve an acceptable midterm result without developing severe dysfunction in the left-sided atrioventricular valve.

Unbalanced atrioventricular septal defect with parachute valve

A 5-month-old male patient presented with right-dominant unbalanced atrioventricular septal defect and left-sided parachute valve, and underwent successful biventricular repair. Because of the presence of a small left ventricle, left atrium, and a single left papillary muscle, an additional orifice was created in the left-sided atrioventricular valve with artificial partitioning of the right-sided atrioventricular valve. There was no evidence of mitral stenosis or regurgitation on follow-up echocardiography.

Pathology and embryology of common atrioventricular canal

This paper focuses on the terminology, classification, pathologic anatomy, embryology, and etiology of common atrioventricular (AV) canal. The designation common AV canal is preferred because it includes both the characteristic septal and leaflet defects; i.e. common AV canal is much more than an AV septal defect, as the cleft in the anterior mitral leaflet indicates. This cleft is indeed a cleft (not a commissure), and the left-sided AV valve is a malformed mitral valve (not a trileaflet non-mitral valve). Common AV canal is classified as complete, partial, transitional, intermediate, balanced, left ventricular type, and right ventricular type. The complete form is subclassified as Rastelli types A, B, and C. The normal embryology of AV canal division is presented in man and in the rat. In humans, the superior and inferior endocardial cushions of the AV canal normally fuse during Streeter’s horizon XVII, i.e. 34–36 days of age. An understanding of the spatial geometry of the endocardial cushions of the AV canal relative to the ventricular and atrial septa helps to explain normal and abnormal development and the classification of common AV canal. Normal morphogenesis is a three-step process. An increase in fibroblast cell-surface adhesiveness may well be important in the morphogenesis of common AV canal in Down syndrome. Etiologically, a single gene stochastic model is now thought more likely than the polygenic multifactorial hypothesis. The Down syndrome congenital heart disease gene (or genes) is (are) now thought to be located on chromosome 21 from q22.1 to q22.3. Common AV canal may be non-syndromic or syndromic, the latter including Down syndrome and the heterotaxy syndromes with asplenia and polysplenia. There are statistically highly significant differences between Down and non-Down canals, and between asplenic and polysplenic canals.

Intraatrial mitral valve insertion with native valve preservation in children

Two patients underwent intraatrial mitral valve insertion for an unsuccessful valvotomy for severe mitral stenosis and left-sided atrioventricular valve insufficiency associated with corrected transposition utilizing a porcine valve from a valved conduit with preservation of the native valve. The valves were inserted using continuous suture distally at the mitral annulus and proximally at the pulled atrial wall distal to the pulmonary veins. Both patients had uneventful hospital course and are doing well at up to 6 months postoperatively. This apporach provides a viable option for congenital mitral stenosis or insufficiency in children.

Congenitally corrected transposition presenting at 24 years: errors of management

AbstractA 24-year-old female without previous symptoms or known heart disease consulted a general practitioner with dyspnoea which had slowly worsened over a period of two years. She was treated for asthma, without investigations, until she presented with rapid atrial fibrillation, chest pain and pulmonary oedema necessitating admission to hospital, where she was found to have “mitral” regur-gitation (of the left-sided atrioventricular valve) and congenitally corrected transposition. At operation the grossly regurgitant left-sided morphologically tricuspid valve was replaced by a “top-hat” inverted aortic valve homograft. The procedure resulted in intermittent heart block, needing a pacemaker, and continued myocardial failure which progressed with new right-sided valvar regurgitation. She died a few months later. Post mortem examination confirmed the double discordance, and showed a fistula around the tricuspid valvar prosthesis, left atrial thrombus and changes in the mitral valve. There was also an unsuspected area of old myocardial ischaemia, associated with severe hypoplasia of the circumflex branch of the right-sided coronary artery. This was considered probably to have been of embolic origin.

Atresia or absence of the left-sided atrioventricular connection in the fetus: echocardiographic diagnosis and outcome.

Our objective was to describe the appearance of atresia of the left-sided atrioventricular valve on the four-chamber view of the fetal heart. Twenty-four cases of atresia of the left-sided atrioventricular connection were detected over a 2-year period. Cases were analyzed according to the appearance of the four-chamber view and the connections of the great arteries. All cases were referred because the left-sided ventricle was smaller than the right. There were five different groups of cardiac anomaly detected. Associated findings varied according to the diagnostic group. It was concluded that it is possible to identify atresia of the left-sided atrioventricular connection accurately during obstetric ultrasound examination by evaluation of the four-chamber view. However, the appearance of this view varies with the diagnosis. Each diagnostic group has different implications in terms of associated extracardiac malformations and postnatal management.

Surgical atrial septostomy without cardiopulmonary bypass

Surgical atrial septostomy using a special atriotomy knife without cardiopulmonary bypass in patients with obstruction of the left-sided atrioventricular valve and complex cardiac anomalies is described. This procedure is effective, safe, and economical for patients in the acute stage after intracardiac repair, and available for patients with a closed fossa ovalis.