Discussion:
HCD is an endemic parasitic infection that can develop in almost any
body part, especially the liver (75%) and lungs (15%).[4]
Echinococci reach the right heart cavities by the portal or lymphatic
routes, reach the left heart through the pulmonary circulation, patent
foramen ovale, or an intracardiac defect that causes a right-left shunt
and settles into the myocardium via the coronary circulation.[5] LV,
the part of the heart with the most abundant blood supply, is most
frequently involved (60%), followed by the right ventricle (10%),
pericardium (7%), pulmonary artery (6%), left atrial appendage (6%)
the interventricular septum (4%) have been reported.[1] In this
case, no focus of HC was detected, except for multiple organ involvement
in the cranial and cardiac areas; accordingly, echinococci probably
reached the left heart without attaching to the pulmonary
capillaries.
HCD is generally asymptomatic, and signs and symptoms typically appear
decades later because the cyst grows slowly.[6]The clinical
presentation of CHC varies depending on the location, age, size,
infection, number, and calcification.[5] Chest pain, palpitations,
and dyspnea are the main symptoms of HCD. However, it can cause fatal
complications such as sudden rupture, suppuration, anaphylactic shock,
arrhythmia, and embolization.[5] HC in the LV are usually located
subepicardially and rarely rupture into the pericardial cavity.[7]
Intracavitary rupture in LV may cause systemic embolism, such as
occlusion of the mesenteric or lower extremity arteries.[8] However,
HC in the right ventricle is usually located subendocardially and causes
pulmonary embolization, mainly due to intracavitary rupture.[9]
Operative and surviving perforated CHCs have been rarely reported in the
literature. In our case, there was occlusion in the distal abdominal
aorta and bilateral iliac arteries due to the rupture of the cardiac
cysts into the LV. The patient had no symptoms, both the CHC and its
complications.
In addition, CHC can mimic left ventricular aneurysms (LVA) or cardiac
malignancies. It can also be thought to cause cardiac aneurysms acquired
in childhood since it is a progressive parasitic infection that forms
cystic cavities. However, no information was found in the literature
regarding this.[10] In our rare case, the evaluations show that the
cyst was not mimicking an aneurysm but caused a true cardiac aneurysm by
the destruction of the left ventricle apical wall that was noticed after
it ruptured.
The diagnosis of cardiac HCD is mainly based on clinical suspicion,
cardiac imaging, and serological tests. Antibody assays are helpful
primarily to confirm the possible radiological diagnosis. However,
negative serological test results do not exclude the
diagnosis.[11]TTE is a very sensitive and specific diagnostic tool
that shows the effect of the lesion on ventricular or valve functions.
CT is necessary in the diagnosis, but it can be misleading, especially
in the case of myocardial cyst rupture. Cardiac MRI can provide valuable
information about the lesion and its relationship to other cardiac and
extracardiac structures.[12] In our patient, the negative
serological tests and the absence of pathological findings in the
abdomen and lung imaging caused a shift away from the diagnosis of HC in
the unit that first evaluated the patient. However, cardiac involvement
was detected by TTE, and MRI revealed that it caused intracavitary
rupture and aneurysm in the apex of the LV.
CHC should be surgically removed even in asymptomatic patients because
of the high fatal risk of complications.[13] Ventricular aneurysms
are also rare in children, and theories of etiology differ.[14]
Approaches to surgical treatment also differ among surgeons.[15]
Typically, aneurysmectomy or primary closure with plication of the
aneurysm sac is preferred.[14] Endoventricular circular patch
plasty, also known as the Dor procedure, was described by Dor in adults
to repair akinetic wall segments due to transmural ischemia. The
procedure reshapes the left ventricle with a suture surrounding the
transition zone between the contractile myocardium and aneurysmal
tissue. It restores ventricular wall continuity with a patch, but the
akinetic or dyskinetic portions of the anterior wall and septum are
excluded from the procedure.[16-17] This surgical approach was
rarely described in children, but it offers the same benefits as in
adults, such as excision of noncontractile (fibrotic) ventricular wall
to minimize akinetic or dyskinetic tissues and restore natural
ventricular chamber geometry.[14-15] In this case, surgical
treatment was deemed appropriate because of our patient’s complicated
CHC, the unknown presence of an intracardiac cystic structure, and the
risks of additional complications related to the apical LVA.
Although some superficially located small cysts can be directly
intervened, resection under cardiopulmonary bypass, since 1962, has been
considered the safest method for its advantages, such as preventing
systemic embolization by placing aortic cross-clamps and enabling the
recognition of additional lesions missed before surgery.[18] Gentle
and limited manipulation of the heart under cardiopulmonary bypass
reduces the risk of operative complications.[19] The choice for
surgical treatment is total excision of the cyst. However, if this is
not possible, complete closure of the cyst by plication and obliteration
of the cavity should be performed since complications are observed in
cases of simple drainage or marsupialization of the cavity near the
cardiac structures.[5] In our case, the LV was explored under
cardiopulmonary bypass,and no pathological tissue sample of the HC was
observed. Surgical repair was performed with aneurysmectomy and Dor
procedure since complete excision would not be possible in the current
state.
Serological and echocardiographic check-ups are recommended five years
after surgical treatment to detect recurrences after manipulation or
cysts not discovered during the operation.[20]No late cardiac
problems or cyst recurrence was observed during the 5 years follow-up
period.