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\begin{document}

\title{Infective endocarditis complicated by rupture of cerebral mycotic
aneurysm in a child with obstructive HCM A case report with review of
the literature}



\author[1]{amel maghrebi}%
\author[2]{wissal skhiri}%
\author[2]{wided gamaoun}%
\author[2]{hela jemni}%
\affil[1]{Fattouma Bourguiba University Hospital of Monastir}%
\affil[2]{Sahloul University Hospital}%


\vspace{-1em}



  
  \date{May 18, 2024}


\begingroup
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\selectlanguage{english}
\begin{abstract}
The literature, infective endocarditis in patients with hypertrophic
cardiomyopathy is virtually limited to isolated reported cases or groups
of cases, therefore the risk of infective endocarditis during HCM
remains poorly defined. It is considered low and mostly limited to
patients with a subaortic obstruction or with an intrinsic mitral defect%
\end{abstract}%



\sloppy


\textbf{Infective endocarditis complicated by rupture of cerebral
mycotic aneurysm in a child with obstructive HCM}

\textbf{A case report with review of the literature}

Amel MAGHREBI\textsuperscript{1} , Wissal SKHIRI\textsuperscript{1} ,
Wided GAMAOUN11Department of Radiology Sahloul University Hospital
Center, Hela JEMNI\textsuperscript{1}

Amel MAGHREBI, MD, Department of Radiology, Sahloul University Hospital
Center, Sousse, Tunisia

Wissal SKHIRI, MD, Department of Radiology, Sahloul University Hospital
Center, Sousse, Tunisia

Wided GAMAOUN, MD, Department of radiology, Sahloul University Hospital
Center, Sousse, Tunisia

Hela JEMNI, PH, Department of radiology, Sahloul University Hospital
Center, Sousse, Tunisia

Corresponding author Amel MAGHREBI (mail: maghrebiamel92@gmail.com)

Amel MAGHREBI\emph{maghrebiamel92@gmail.com}, collecting data and
writing original draft

Wissal SKHIRI,\emph{wissalskhiri28@gmail.com}, investigation

Wided GAMAOUN,\emph{gamaounwided@yahoo.fr} , validation, supervision

Hela JEMNI,\emph{helajemni@gmail.com} , supervision

Please note : Written informed consent was obtained from parents of the
patient to publish this report in accordance with the journal`s patient
consent policy

We declare no conflict of interest

\textbf{Abstract}

Left ventricular (LV) hypertrophy is defined by a thickness
\textgreater{} 15 mm in the adult index case or \textgreater{} 13 mm in
the adult related case {[}1{]}

In children, it is defined according to a nomogram based on age and body
surface area. Hypertrophy is usually asymmetrical predominating over the
interventricular septum. {[}1{]}

The literature {[}2,3{]} , infective endocarditis in patients with
hypertrophic cardiomyopathy (HCM) is virtually limited to isolated
reported cases or groups of cases, therefore the risk of infective
endocarditis during HCM remains poorly defined and thought to be low
because HCM is a disorder of the myocardium and not the
endocardium.{[}4{]}

Spirito et al{[}1{]} assessed the occurrence of IE in HCM was 4.3\% at
10 years and discerned an HCM subgroup with both obstruction and atrial
dilatation prone to develop IE {[}5{]}

It is considered low and mostly limited to patients with a subaortic
obstruction at rest and or with an intrinsic mitral defect. {[}2{]}

The site of the vegetation is usually the anterior leaflet of the mitral
valve but vegetations have been reported to occur at the septal level
(area of contact with the mitral) and or on the valve {[}2{]}.

We present a case of infective endocarditis on culture-negative native
mitral valve in a previously asymptomatic 10-year-old child with HCM.

This case is not only a rare pediatric HCM with IE, but it also
demonstrates the invaluable role of imaging in the diagnosis of HCM and
its complications, especially in the case of IE.

\textbf{Key words~:}

Obstructive, hypertrophic cardiomyopathy, mitral endocarditis, cerebral
mycotic aneurysm, complicated

\textbf{INTRODUCTION}

Infective endocarditis complicating HCM is a rare entity with a
\textless{}5\% 10-year risk of disease progression {[}1{]}.

The incidence of IE among HCM patients has been described to be 18 to 28
times higher than in the general population and left ventricular outflow
tract obstruction (LVOTO) and enlarged left atria have been reported as
factors

that increase the risk of IE in HCM {[}6{]}

Prognosis is worse in patients with IE associated with HOCM than in
patients with IE alone or associated with congenital heart disease
{[}6{]}

This report describes a pediatric case of HOCM with IE and its cerebral
complication

\textbf{Case presentation}

A 10-year-old child with no particular history was admitted to the
infectious diseases department for prolonged fever and dyspnea.

On examination he had a temperature of 38.5\selectlanguage{ngerman}°C and a systolic murmur at
the mitral focus

EKG: sinus tachycardia at 150bpm, systolic left ventricular hypertrophy
(figure 1)\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image1/image1}

\end{center}
\end{figure}

\textbf{Figure 1~: left ventricular hypertrophy with repolarization
disorder and biphasic T wave}

On biology: he had a biological inflammatory syndrome with white blood
cells at 22000 and CRP 80 with negative blood cultures.

A chest X-ray showed cardiomegaly

A trans thoracic echography showed a left ventricle of low compliance
with a parietal hypertrophy located at the level of the basal and medial
septum mesuring 21mm (figure 2 and 3)\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image2/image2}

\end{center}
\end{figure}

\textbf{Figure 2~: transthoracic ultrasound centered on the left
ventricule (longitudinal view) showing asymmetrical thickening of the
left ventricular wall, especially the septum (arrow)}

LVEF at 75\%.

Voluminous mobile vegetations mutilating the anterior mitral valve
measuring a total of 40mm long axis prolapsing into the OG which is
dilated with significant mitral insufficiency. (figure 3 and 4)

On the aortic pathway: a septal hypertrophy obstruction measuring 27mm
at this level.\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image3/image3}

\end{center}
\end{figure}

\textbf{Figure 3~: transthoracic ultrasound centered on the left
ventricule (longitudinal a and axial b view in the mitral level) showing
vegetation (arrow) on the anterior leaflet of mitral valve}\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image4/image4}

\end{center}
\end{figure}

\textbf{Figure 4~: Pulsed doppler shows significant regurgitation}

The patient was treated with ampicillin, oxacillin and gentamicin.

A thoraco abdomino pelvic and cerebral CT scan was performed to assess
the extent of the infection.

No notable complication or secondary infectious location was found.

Below are the sections centered on the heart. (figure 5)\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image5/image5}

\end{center}
\end{figure}

\textbf{Figure 5~: Reconstructions of the heart~: short axis (a) and
sagittal oblique showing the hunting chamber (b)}

\textbf{We note the important thickness of the septal wall at 26mm,
(arrow) the reduction of caliber of the LV and the hunting chamber
(star) with dilatation of left atrium (arrow head)}

The patient was operated on day 3 of his admission with mitral valve
replacement + septal myomectomy

Then he was put on an intravenous antibiotic treatment (imipenem,
teicoplanin and gentamicin)

The evolution was marked by an improvement with apyrexia, regression of
the biological inflammatory syndrome and disappearance of the sub-aortic
gradient and mitral insufficiency.

During the follow-up :

7 days after the operation, the patient presented a convulsive seizures.

A cerebral CT scan was performed, it showed a cerebral hematoma with
quadri ventricular flooding, sub arachnoid hemorrhage and a diffuse
cerebral edema responsible for cerebral herniation; sub falcoriel,
central, temporal (figure 6).\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image6/image6}

\end{center}
\end{figure}

\textbf{Figure 6~: cerebral CT scan showing~: left frontotemporal
hematoma (star) with quadri ventricular and sub arachnoidal hemorrhage
(arrow head, b,c) with subfalcine (arrow, c), homolateral uncal
transtentorial (arrow, a) cerebral herniations}\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image7/image7}

\end{center}
\end{figure}

\textbf{Figure 7~: cerebral CT arteriography , axial plane (a) and
reconstruction in the frontal plane with MINMIP (b) showing a saccular
aneurysm of the left sylvian artery (arrow)}

Arterial phase of CT arteriography was done and showed a ruptured
saccular aneurysm of the left middle cerebral artery M2 .(Figure 7)

\textbf{Discussion}

Infective endocarditis in hypertrophic cardiomyopathy is a serious
complication associated with high morbidity and mortality.{[}1{]}

Although it is not common, it has been reported in the literature, but
the data are limited to single cases or groups of cases.

The most cited study was that of Spirito and colleagues who followed the
evolution of 810 patients with HCM and the incidence was reported to be
1.4 cases/person/year and the main risk factors were outlet obstruction
and OG dilatation {[}2{]}.

Another chinese study of Wang et al {[}7{]} showed that the proportion
of HCM patients with IE was 0.19\%,with the estimated incidence of
0.15/1 000 person-years in HCM patients.

The mitral valve was the most affected in studies conducted by Spirito
et al in 1999 and Fernando et al in 2013 with a percentage of 70 and
71\% respectively {[}2{]}

An obstructive HCM was present in all cases of infective endocarditis
{[}10{]} with a statistically significant association between
obstruction and infective endocarditis in the study of Spirito et al
{[}2{]} but the multicenter, prospective, cohort study of Dominguez et
al {[}3{]} and Sims et al {[}8{]} found a similar incidence of IE with
or without left ventricular outflow tract obstruction (LVOTO).
Nevertheless, the small sample size could be just a statistical outlier,
limiting its universality . In addition, the latter study found no
clinical difference between infective endocarditis in HCM patients with
and without obstruction..

Because HCM is a disorder of the myocardium and not the endocardium, it
has been considered to have low risk of IE. In fact the occurrence of
infective endocarditis in HCM patients can be explained by endocardial
lesions secondary to turbulent flow during ejection and contact between
the anterior leaflet of the mitral valve and the septum during systole
which is called \selectlanguage{ngerman}«~Venturi effect~» , thus direct trauma resulting from
septal-anterior mitral leaflet contact may predispose to infection and
vegetation formation {[}2,4{]}

We note that the vegetation in our case was indeed located on the
anterior leaflet of mitral valve.

The most common germs found in the literature were staphylococcus aureus
and streptococcus , with this latest being more found among native-valve
HCM patients {[}2, 3{]}.

The germ in our case was not identified with blood culture remaining
negtive.

Mitral valve replacement surgery and septal myomectomy are the two
accepted therapeutic methods for symptomatic obstructive HCM refractory
to medical treatment but the larger series on the surgical treatment of
HCM suggests that the two operations are rarely combined {[}5,6{]}.

We recall that our patient had both operations at the same time.

The occurrence of a complication such as rupture of an intracerebral
mycotic aneurysm with hematoma, as was the case in our patient, is very
rare and limited to a few isolated cases reported in the literature
{[}7, 8{]}, in a recent study of Jason R Sims et al {[}8{]}, symptomatic
embolic complications occurred in 33\% of cases.

\textbf{Conclusion}

Infective endocarditis is an uncommon but serious complication in HCM
that can occur in patients with or without LVOTO. Septic embolization
and valve lesions are common in these patients. IE patients with HCM
might have a poor prognosis compared to those without HCM and should
receive cardiac surgery as early as possible.

Mortality is high but similar to that found in patients with or without
indication for endocarditis prophylaxis. However, predisposing factors
and Streptococci infections are more frequent among native-valve HCM
patients.

\textbf{Références}

1. Maron BJ, Maron MS. Hypertrophic cardiomyopathy. Lancet. 19 janv
2013;381(9862):242\selectlanguage{english}-55.

2. Spirito P, Rapezzi C, Bellone P, Betocchi S, Autore C, Conte MR, et
al. Infective endocarditis in hypertrophic cardiomyopathy: prevalence,
incidence, and indications for antibiotic prophylaxis. Circulation. 27
avr 1999;99(16):2132\selectlanguage{english}-7.

3. Dominguez F, Ramos A, Bouza E, Munoz P, Valerio MC, Farinas MC, et
al. Infective endocarditis in hypertrophic cardiomyopathy: A
multicenter, prospective, cohort study. Medicine. juin
2016;95(26):e4008.

4. Chen M. Infective endocarditis in hypertrophic obstructive
cardiomyopathy. J Clin Ultrasound. nov 1992;20(9):612\selectlanguage{english}-4.

5. Wu ZW, Yan H. Infective endocarditis in obstructive hypertrophic
cardiomyopathy: a case series and literature review. Chinese Medical
Journal. 5 mai 2021;134(9):1125\selectlanguage{english}-6.

6. Alessandri N, Pannarale G, del~Monte F, Moretti F, Marino B, Reale A.
Hypertrophic obstructive cardiomyopathy and infective endocarditis: a
report of seven cases and a review of the literature. Eur Heart J. nov
1990;11(11):1041\selectlanguage{english}-8.

7. Wang P, Song L, Gao XJ, Wang SY, Song YH, Qiao SB. {[}Clinical
analysis of 14 infective endocarditis in patients with obstructive
hypertrophic cardiomyopathy{]}. Zhonghua Nei Ke Za Zhi. 1 dec
2020;59(12):982\selectlanguage{english}-6.

8. Sims JR, Anavekar NS, Bhatia S, O'Horo JC, Geske JB, Chandrasekaran
K, et al. Clinical, Radiographic, and Microbiologic Features of
Infective Endocarditis in Patients With Hypertrophic Cardiomyopathy. Am
J Cardiol. 15 fevr 2018;121(4):480\selectlanguage{english}-4.

9. ten~Berg JM, Suttorp MJ, Knaepen PJ, Ernst SM, Vermeulen FE, Jaarsma
W. Hypertrophic obstructive cardiomyopathy. Initial results and
long-term follow-up after Morrow septal myectomy. Circulation. oct
1994;90(4):1781\selectlanguage{english}-5.

10. Robbins RC, Stinson EB. Long-term results of left ventricular
myotomy and myectomy for obstructive hypertrophic cardiomyopathy. J
Thorac Cardiovasc Surg. mars 1996;111(3):586\selectlanguage{english}-94.

11. Anguita M, Romo E, Vinals M, Ostos J, Bueno G, Vivancos R, et al.
{[}The management by medical treatment of an intracranial mycotic
aneurysm in a patient with infectious endocarditis with negative blood
cultures and hypertrophic myocardiopathy{]}. Rev Esp Cardiol. oct
1991;44(8):556\selectlanguage{english}-9.

12. Inoue T, Shinohara T, Saga T. Surgical treatment of infective
endocarditis complicated by intracranial hemorrhage in a patient with
hypertrophic obstructive cardiomyopathy. Can J Cardiol. 1 mai
2004;20(6):643\selectlanguage{english}-5.

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