Article Type : Case Report
Authors : Mleyhi S, Ben Saida F, Saad A, Ben Mrad M, Ben Omrane S, Kalfat T and Denguir R
Keywords : Echinococcus Granulosis; Cardiac failure; Rupture; Emergency; Recidivism; Surgery; Albendazole
Hydatidosis
is an endemic disease in Tunisia. The hepatic and pulmonary localizations are
the most frequent. Diagnosis is often late due to lack of specificity and
clinical latency which characterize this affection. Cardiac involvement is rare
and may pose a diagnostic and therapeutic problem due to recidivism. We report
a case of massive invasive intracardic and pericardial hydatidosis in a
37-year-old male operated for hydatic liver cyst at the age of 24. The patient
was operated initially since 5 years in emergency under cardiopulmonary bypass
for multiple cardio pericardial hydatidosis discovered incidentally,
threatening both the arterial and venous systems with uneventful post-operative
course. Reconsult after 6 years for shortness of breath, palpitation and
features of congestive cardiac failure related to relapse of the hydatid
desease redisseminating in cardiac cavities. A thoracic magnetic resonance
imaging (MRI) showed a recurrence of the cardiopericardial hydatidosis with
pulmonary artery hypertension (PAH) and multiple septic and arterial hydatic
emboli. The patient was treated medically by anthelmintic medecines
(albendazole) and biltricide (praziquantel) with a good clinical evolution and
stable cardiac echography controls.
The hydatid cyst is a parasitic tumor caused by
Echinococcus granulosus in an intermediate host. Although cardiac involvement
is a rare manifestation of hydatid cyst disease, its early diagnosis and
surgical management are crucial. Patients with cardiac hydatidosis may develop
acute life-threatening complications secondary to their invasion of surrounding
cardiac structures, such as cyst rupture together with systemic and pulmonary
dissemination.
A 37-year-old man of rural origin, who had been operated
for a hydatic liver cyst at the age of 24. The patient reconsulted after 5
years for multiple cardiopericardial hydatidosis, right and left ventricle
hydatid cysts, also in the right atrium and the aorta. The patient underwent a
cardiac surgery under cardiopulmonary bypass. A right ventricle cyst of 7 cm is
discovered in intraoperative and developed on the lower surface. A left
ventricle cyst of 6cm , a cyst of the right atrium measuring 2cm near the
inferior vena cava and , a cyst of the wall of the aorta of 3 cm at the level
of its root compressing the superior vena cava with a multitude of small
disseminating cysts all over the territory of the pericardium with secondary
pericardial hydatidosis. After setting up of protective fields with hypertonic
saline solution, the different cavities were approached to remove the hydatic
membranes and carefully clean the pericardial cavity with hypertonic saline
solution. Cardiopulmonary bypass lasted 85 minutes and the postoperative course
was uneventful. During this period he was treated by albendazole with good
observance and tolerance with a cardiac ultrasound control showing
disappearance of oval filing of the right atrium and since the patient is
followed in the infectious diseases department for 2 years then lost sight for
5 years. Currently the patient has been hospitalized in our reanimation unit of
the cardiovascular surgery department for shortness of breath and palpitation.
He had been unwell for the past 2 years with complaints of easy fatigability, progressive
breathlessness. On examination, he had features of congestive heart failure
with distended jugular veins, bilateral basal crepitations, hepatomegaly and
pedal edema. His heart rate was 110, blood pressure 90/50 mmHg. Biological
assessment has objected blood eosinophilia at 1900/mm3.Chest x-ray showed
cardiomegaly and left apical opacity. Cardiac ultrasound revealed left
ventricle ejection fraction at 60%, pulmonary arterial hypertension 60 mmhg,
hyperechogenic cyst mesuring 5*2 cm adjacent to the roof of the right atrium
overlapping tricuspid. The pulmonary pathway is free and presence of 3 adjacent
hyperechogenic cystic images of the pericardium behind the left ventricle. A
thoracic magnetic resonance imaging(MRI) angiography showed cystic formations
hypointense on T1-weighted images and hyperintense on T2-weighted at: left
ventricular lateral wall 9*3 cm , right atrium inracavitar 4*2cm , pericardium
next to the left ventricular tip 4* 3.5 cm and ascending aorta 3* 2,5 cm , dilatation of the pulmonary artery and infundibulum and alteration of the
function of the left ventricle to 36% with bilateral arterial hydatic
emboli.The diagnosis retained was a recidive of the cardiopericardial hydatidosis with pulmonary artery
hypertension(PAH) and multiple septic and arterial hydatic emboli. The patient
was treated medically by anthelmintic medecines and biltricide with a good
clinical evolution and weaning out of oxygen, SpO2 at 95% at ambient air.
Surgical abstinence was decided since the stability of the lesions .Then the
patient had cardiac ultrasound control of residual hydatic cysts showing steady
appearance of lesions with good hemodynamic tolerance.
Hydatid disease is commonly caused by the parasite
E.granulosus. Humans usually become affected by the ingestion of food or water
contaminated by dog faeces containing the eggs. Hydatid cysts are most common
in the liver and lungs of humans. Cardiac hydatid cysts are rare, and cardiac
echinococcosis is about 0.5–2% of all hydatid cases [1-3]. Usually the cysts
are located primarily within the myocardial layers or secondarily in the
pericardium. Intracavitary cyst locations are rarely observed. The left and
right ventricles, pericardium, pulmonary artery, left atrial appendage, and
interventricular and interatrial septum’s can be involved. Cardiac
echinococcosis is generally asymptomatic. Its clinical features depend on the
size site, and stage of the cyst and the presence of haemodynamic consequences.
The cysts may cause arrhythmias, acute coronary syndrome, cardiac tamponade, or
congestive cardiac failure as seen in our case [4, 5]. In our case, the patient
had multiple and invasive cardiac cysts. The pericardium was invaded with small
disseminating cysts all over its territory probably related to secondary
pericardial hydatidosis. In fact pericardial location may occur (4% to 10%) and
it is as a result of rupture of superficially located myocardial cysts or
spreading of the contents during prior surgical removal. Expansion of cysts is
a common manifestation of right ventricular cysts. Unfortunately, in our case
some of cysts were found growing over the tricuspid valve .The chest radiograph
can be normal or usually shows a cardiomegaly, as in our patient.
Calcifications may be noted also in the outer pericyst. Hydatidosis has
specific aspect demonstrated on echocardiography, multiloculated image, and
presence of small daughter cysts or thin floating membranes. The haemodynamic
consequences of the lesion can also be assessed. But in our case the left
ventricular function was falsely reassuring.The hydatid cyst usually has a
characteristic appearance on MRI: an oval lesion that is hypointense on
T1-weighted images and hyperintense on T2-weighted images. A typical Finding on
T2-weighted images is a hypointense peripheral ring, which represents the
pericyst (a dense fibrous capsule from the reactive host tissue). The
multivesicular nature of the cystic mass and membrane detachment indicate the
true diagnosis. The cysts may be single or multiple uniloculated or
multiloculated, and thin or thick walled. Calcification of the cyst wall is a
more specific sign, as well as presence of daughter cysts, and membrane
detachment [6-8]. Surgical intervention for cardiac hydatidosis is the
definitive treatment, even in asymptomatic patients to prevent complications
(such as rupture or embolism), followed by anthelmintic drugs (benzimidazoles)
to prevent recurrence [9]. The surgical approach depends on the location,
number, and of the size of cysts. In our case, due to presence of multiple
intracardiac and pericardial cysts, the patient was operated under
cardiopulmonary bypass. Cardiopulmonary bypass is crucial to prevent rupture,
embolism, and anaphylaxis. Localization of cysts in Right cardiac cavities like
in our case may be dangerous, due to the low pressure regime of the right
cavities and the elective cyst development in sub-endocardial, this one ends up
breaking in traffic lung (30% mortality) [10]. The distribution of cystic
locations is parallel to the importance of coronary blood flow: 60% in the left
ventricle, 15% in the right ventricle. The use of albendazole, with or without
praziquantel, has shown to be beneficial like in our case In fact his
effectiveness of benzimidazoles appears to be more dependent on the duration of
treatment than drug blood levels. We report this case first, to ensure the
importance of a good preoperative evaluation of multiple intracardiac
localizations of hydatid cyst to guide cardiac surgery as well as to prevent
complications and secondary to focus on medical treatment that should be
reinitiated and maintained indefinitely to remain stable on pre-and
post-operative, in the case we reported the patient was lost sight for a long
period after surgery and did not take his drugs.
Cardiac hydatid is a very rare manifestation of
hydatid disease. The diagnosis is suggested by the identification of daughter
cysts in a multiseptated lesion, presence of hydatid sand, wall calcifications
and the spokewheel appearance. Echocardiography is the modality of choice for
the diagnosis of cardiac hydatid cysts, owing to its widespread availability,
and the ability to analyse concurrent haemodynamic consequences. MRI may aid in
accurate localization of the lesion, detection of multiple lesions, study of
concurrent lung lesions and detection of multiorgan involvement, as well as
differential diagnosis and follow-up.