Concomitant septal myectomy and mitral valve repair in a child with Noonan syndrome

Q: What is the clinical evidence from this study?

Study design: Case Report. Population: Noonan syndrome with hypertrophic obstructive cardiomyopathy and severe mitral regurgitation (n=1). Intervention: Concomitant surgical septal myectomy (Morrow's procedure) and mitral valve leaflet retention repair. Primary outcome: Postoperative left ventricular outflow tract (LVOT) pressure gradient, systolic anterior motion (SAM), and mitral regurgitation (MR) grade.

Q: What does this research mean for the field?

Concomitant septal myectomy and mitral valve repair relieves left ventricular outflow tract obstruction and reduces mitral regurgitation in pediatric patients with Noonan syndrome and hypertrophic obstructive cardiomyopathy. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

Toshio Doi; Daisuke Toritsuka; Akihiko Higashida; Shigeki Yokoyama; Kazuaki Fukahara; Naoki Yoshimura

doi:10.1002/ccr3.3531

November 18, 2020Clinical Case ReportsOpen Access

Concomitant septal myectomy and mitral valve repair in a child with Noonan syndrome

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Key result

Concomitant septal myectomy and mitral repair reduces LVOT gradient by ~57 mmHg in Noonan syndrome.

n=1

Figures

Graphical abstract published by Clinical Case Reports — Graphical abstract · Published by Clinical Case Reports · CC BY

Why the study?

Although septal myectomy is the gold standard treatment for drug-refractory hypertrophic obstructive cardiomyopathy, few reports detail the surgical outcomes in pediatric patients with Noonan syndrome, and little has been reported on its relationship with mitral valve disease.

Does concomitant surgical septal myectomy and mitral valve leaflet retention repair improve left ventricular outflow tract obstruction and severe mitral regurgitation in a pediatric patient with Noonan syndrome and hypertrophic obstructive cardiomyopathy?

Key Points

To report the surgical management of drug-refractory hypertrophic obstructive cardiomyopathy and severe mitral regurgitation in a pediatric patient with Noonan syndrome.
A 7-year-old boy with Noonan syndrome, a RAF1 mutation, and worsening left ventricular outflow tract obstruction despite bisoprolol and cibenzoline underwent open-heart surgery.
Surgical intervention consisted of transaortic resection of a 10 × 15 × 5 mm segment of the ventricular septum (Morrow procedure) combined with mitral valve leaflet retention repair via left atrial incision.
The left ventricular outflow tract pressure gradient decreased from 64 mm Hg preoperatively to 7 mm Hg postoperatively, with resolution of systolic anterior motion and reduction to trace mitral regurgitation.
Postoperative recovery was complicated by atrial fibrillation and ventricular tachycardia that stabilized with amiodarone, and the patient was discharged walking 47 days after surgery.

Study Design

Type

Case Report (n=1)

Multicenter

No

Structured PICO

Does concomitant surgical septal myectomy and mitral valve leaflet retention repair improve left ventricular outflow tract obstruction and severe mitral regurgitation in a pediatric patient with Noonan syndrome and hypertrophic obstructive cardiomyopathy?

P

Population

A 7-year-old boy with Noonan syndrome (RAF1 mutation), drug-refractory hypertrophic obstructive cardiomyopathy (LVOT gradient 64 mm Hg), and severe mitral regurgitation due to systolic anterior motion, followed until hospital discharge at 47 days post-surgery.

I

Intervention

Concomitant transaortic surgical septal myectomy (Morrow's procedure, resecting a 10 x 15 x 5 mm ventricular septum segment) and mitral valve leaflet retention repair (anterior leaflet sutured to the posterior annulus near both fibrous trigons using two 5-0 polypropylene sutures with autologous pericardial pledgets) via full median sternotomy.

O

Outcome

Postoperative left ventricular outflow tract (LVOT) pressure gradient, resolution of systolic anterior motion (SAM), and reduction in mitral regurgitation (MR) grade on transthoracic echocardiography prior to discharge (47 days post-surgery).surrogate

Concomitant mitral valve leaflet retention repair alongside septal myectomy effectively resolves both severe LVOT obstruction and SAM-related mitral regurgitation when intrinsic mitral leaflet degeneration is present in pediatric Noonan syndrome-associated HOCM.

Implication

Hypothesis-generating for combined myectomy and valve repair in pediatric Noonan HOCM; should not change practice without further evidence.

Abstract

We successfully performed concomitant septal myectomy and mitral valve repair in a child with Noonan syndrome.

Discussion

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Cite This Study

Doi et al. (2020) conducted a case report in Noonan syndrome with hypertrophic obstructive cardiomyopathy and severe mitral regurgitation (n=1). Concomitant surgical septal myectomy (Morrow's procedure) and mitral valve leaflet retention repair was evaluated on Postoperative left ventricular outflow tract (LVOT) pressure gradient, systolic anterior motion (SAM), and mitral regurgitation (MR) grade. Concomitant septal myectomy and mitral valve repair reduced the LVOT gradient from 64 mm Hg to 7 mm Hg, resolved SAM, and reduced MR to trace in a child with Noonan syndrome.

synapsesocial.com/papers/6a7465043e32726c44092eff https://doi.org/10.1002/ccr3.3531

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Concomitant septal myectomy and mitral valve repair in a child with Noonan syndrome

Figures

Key Points

Study Design

Structured PICO

Implication

Abstract

Discussion

Cite This Study

Concomitant septal myectomy and mitral valve repair in a child with Noonan syndrome

Figures

Key Points

Study Design

Structured PICO

Implication

Abstract

Discussion

Cite This Study

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