Clinical characteristics and diagnosis of double-orifice left atrioventricular valve associated with other congenital heart disease

Objective: The purpose of this study was to determine whether Real-time three-dimensional echocardiography (RT3D) is superior to two-dimensional echocardiography (2D) in determining mechanisms and site(s) of atrioventricular valve (AV valve) regurgitation in congenital heart disease. Background: Data is lacking on the utility of RT3D echo in congenital abnormalities of the AV valves. Methods: Between May 2006 and April 2007, 35 cases were prospectively studied prior to AV valve repair (20 left and 15 right) by 2D transthoracic (2DTTE), 2D transesophageal (2DTEE) and RT3D (Philips matrix array X 3–1 and X 7–2). Thirty one had significant and 4 mild regurgitation. Ages ranged from 24 days to 30 years (mean 13.8 years), weight 2.2 to 42kg (mean 26.1Kg). The 2DTTE and TEE were reviewed by a blinded observer. The 3D data were analyzed by a separate observer. In 28 patients surgical findings and regurgitation from saline testing were recorded by digital video and later analyzed by an experienced cardiac surgeon. The remaining 7 had a detailed description of the valve morphology documented immediately after surgery. Surgical findings were used as the gold standard for the assessment of structural abnormalities, with RT3D for the evaluation of regurgitation. Results: See table for structural abnormalities. For both the left and right AV valve, saline testing provided a poorer correlation with RT3D color Doppler jet location, in particular for regurgitant jets from the commmissures (p < 0.01). Conclusion: Transthoracic RT3D provides new and superior information as to the mechanisms and site(s) of AV valve failure in congenital heart disease. Concordance between 2D TTE, 2D TEE and 3D TTE compared to surgical findings

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Improvement in ventricular function with rhythm control of atrial arrhythmias may delay the need for atrioventricular valve surgery in adults with congenital heart disease

Congenital Heart Disease ◽

10.1111/chd.12833 ◽

2019 ◽

Vol 14 (6) ◽

pp. 931-938

Author(s):

Benjamin Zielonka ◽

Yuli Y. Kim ◽

Gregory E. Supple ◽

Sara L. Partington ◽

Emily S. Ruckdeschel ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Ventricular Function ◽

Congenital Heart ◽

Rhythm Control ◽

Valve Surgery ◽

Atrial Arrhythmias ◽

Atrioventricular Valve

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Clinical Characteristics of Necrotizing Enterocolitis in Preterm Patients With and Without Persistent Ductus Arteriosus and in Patients With Congenital Heart Disease

Frontiers in Pediatrics ◽

10.3389/fped.2020.00257 ◽

2020 ◽

Vol 8 ◽

Author(s):

Sonja Diez ◽

Lea Tielesch ◽

Christel Weiss ◽

Julia Halbfass ◽

Hanna Müller ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Necrotizing Enterocolitis ◽

Clinical Characteristics ◽

Congenital Heart ◽

Ductus Arteriosus ◽

Persistent Ductus Arteriosus

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Outcomes and Prognostic Factors for Adult Patients With Congenital Heart Disease Undergoing Primary or Reoperative Systemic Atrioventricular Valve Surgery

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135117692974 ◽

2017 ◽

Vol 8 (3) ◽

pp. 346-353

Author(s):

Elizabeth H. Stephens ◽

Jiho Han ◽

Jonathan Ginns ◽

Marlon Rosenbaum ◽

Paul Chai ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Prognostic Factors ◽

Adverse Events ◽

Congenital Heart ◽

Atrioventricular Septal Defect ◽

The Other ◽

Mitral Valve Stenosis ◽

Atrioventricular Valve ◽

Exact Test

Background: Adults with congenital heart disease (ACHD) undergoing systemic atrioventricular valve (SAVV) surgery are a complex, understudied population. We assessed midterm outcomes and prognostic factors in ACHD undergoing SAVV surgery. Methods: We performed retrospective evaluation of ACHD undergoing SAVV surgery from January 2005 to February 2016: 14 (33%) patients with congenital mitral valve stenosis/regurgitation, 15 (35%) with atrioventricular septal defect (AVSD), and 14 (33%) with congenitally corrected transposition of the great arteries (ccTGA) with systemic tricuspid valve regurgitation. Adverse events were defined as mortality, reoperation on SAVV, and late more-than-moderate (> moderate) SAVV regurgitation. Statistical analysis was performed using Fisher’s exact test and one-way analysis of variance as well as univariate and multivariate risk factor analysis. Results: Fifteen (35%) patients had preoperative systemic ventricular dysfunction, including 13 patients with ccTGA (93%, P < .001). Twenty-three (54%) patients underwent valve repair, 20 (47%) patients underwent replacement, and 20 (47%) patients underwent an associated procedure. Replacement was higher in patients with ccTGA (86%) than the other groups ( P < 0.01). Thirty-seven patients (91% of survivors) were free of significant SAVV regurgitation at last follow-up, with patients with AVSD having greater regurgitation grades compared to the other groups ( P < 0.01). In-hospital mortality, late mortality, late > moderate SAVV regurgitation, and SAVV reoperation rates were 5% (n = 2), 2% (n = 1), 9% (n = 3), and 7% (n = 3), respectively. On multivariate analysis, predischarge SAVV regurgitation grade was the only significant predictor of adverse events (odds ratio = 8.2, 95% confidence interval: 1.1-63.8, P = .045). Conclusion: Overall outcomes in this challenging population are good. The single factor associated with adverse events was predischarge SAVV regurgitation grade.

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