Submitting/Presenting Author

Bianca CherestalFollow

Presenter Status

Fellow

Abstract Type

Case report

Primary Mentor

Doaa Aly, MD

Start Date

12-5-2021 11:30 AM

End Date

12-5-2021 1:30 PM

Presentation Type

Poster Presentation

Description

Background: Congenital mitral regurgitation is a rare condition and can be challenging to manage when presenting in the neonatal period

Objectives/Goal: Two week old male presented with poor weight gain, murmur and cardiomegaly on chest X-ray. Echocardiogram showed moderate to severe mitral regurgitation (MR) and suprasystemic pulmonary hypertension (PHN) (fig 1 a, b). The mitral valve (MV) leaflets were thickened and tethered with failure of central coaptation. PHN was classified as WHO I and II (due to persistent PHN of newborn and MR respectively). Inhaled nitric oxide, Enalapril and Furosemide were initiated. Cardiac catheterization revealed PVRi of 8.9 WU x m2 and CT was non-specific for lung parenchymal disease. Sildenafil and Flolan were added to reverse PHN prior to proceeding with MV repair. At 4 weeks of age he underwent mitral valvuloplasty which was complicated by severe MR and left heart failure (fig 1 c-d). Successful MV replacement with 17 mm St Jude mechanical valve was performed at 11 weeks (fig 1 e). PHN medications were weaned and patient is now ready for discharge.

Methods/Design: Patient presented with severe left heart failure and PHN secondary to severe congenital MR. MV intervention was indicated due to failed medical management. While MV replacement, can be a challenge, it was ultimately necessary given the severe post repair residual regurgitation.

Results:

Conclusions: This case highlights the complexity of decision making for congenital MR, and the role of MV replacement in the case of failed repair.

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May 12th, 11:30 AM May 12th, 1:30 PM

Congenital mitral valve regurgitation, the dilemma of repair vs replacement.

Background: Congenital mitral regurgitation is a rare condition and can be challenging to manage when presenting in the neonatal period

Objectives/Goal: Two week old male presented with poor weight gain, murmur and cardiomegaly on chest X-ray. Echocardiogram showed moderate to severe mitral regurgitation (MR) and suprasystemic pulmonary hypertension (PHN) (fig 1 a, b). The mitral valve (MV) leaflets were thickened and tethered with failure of central coaptation. PHN was classified as WHO I and II (due to persistent PHN of newborn and MR respectively). Inhaled nitric oxide, Enalapril and Furosemide were initiated. Cardiac catheterization revealed PVRi of 8.9 WU x m2 and CT was non-specific for lung parenchymal disease. Sildenafil and Flolan were added to reverse PHN prior to proceeding with MV repair. At 4 weeks of age he underwent mitral valvuloplasty which was complicated by severe MR and left heart failure (fig 1 c-d). Successful MV replacement with 17 mm St Jude mechanical valve was performed at 11 weeks (fig 1 e). PHN medications were weaned and patient is now ready for discharge.

Methods/Design: Patient presented with severe left heart failure and PHN secondary to severe congenital MR. MV intervention was indicated due to failed medical management. While MV replacement, can be a challenge, it was ultimately necessary given the severe post repair residual regurgitation.

Results:

Conclusions: This case highlights the complexity of decision making for congenital MR, and the role of MV replacement in the case of failed repair.