WADY WRODZONE
Congenital pericardial defect with Gerbode type
septal defect in rotated heart: report of a case
Ubytek worka osierdziowego oraz ubytek w przegrodzie
międzykomorowej typu Gerbode w zrotowanym sercu opis przypadku
Ireneusz Haponiuk, Maciej Chojnicki, Radosław Jaworski, Mariusz Sroka, Mariusz Steffek,
Piotr Czauderna
Oddział Kardiochirurgii Dziecięcej Pomorskiego Centrum Traumatologii im. Mikołaja Kopernika w Gdańsku
Kardiochirurgia i Torakochirurgia Polska 2010; 7 (3): 276 279
Abstract Streszczenie
Asymptomatic pericardial defects are rare and found mostly Bezobjawowe wrodzone ubytki osierdzia rozpoznaje się prze-
incidentally during cardiac surgery. In one third of all cases ważnie przypadkowo podczas zabiegów kardiochirurgicznych.
absence of pericardium accompanies various congenital heart W ok. 30% przypadków ubytkom osierdzia towarzyszą inne
defects, and is diagnosed intraoperatively. We present a case wrodzone wady serca. W pracy zaprezentowano przypadek
of an 8-month-old male infant with Gerbode-type septal de- 8-miesięcznego chłopca z ubytkiem w przegrodzie międzyko-
fect (LV-RA communication) with rightward rotation of the morowej typu Gerbode (komunikacja lewa komora prawy
heart and left-sided partial pericardial defect, with pericardial przedsionek) w zrotowanym sercu z historią przewlekłych infek-
adhesions and symptoms of left-sided pressure pneumotho- cji dróg oddechowych. W trakcie zabiegu operacyjnego korekcji
rax while opening the pericardial sac. The unique co-existence wady serca stwierdzono zrosty osierdziowe oraz lewostronną
of Gerbode septal communication and position anomaly of the odmę opłucnową. Powstała ona po otwarciu worka osierdzio-
heart with congenital defect of the pericardium caused the ef- wego w mechanizmie pułapki powietrznej przez istniejący uby-
fect of valvular air-trapping mechanism in the area of pericar- tek osierdzia w okolicy uszka lewego przedsionka. Zrosty osier-
dial wall discontinuity that needed a change of the operative dziowe mogły powstać na skutek komunikacji jamy opłucnowej
strategy prior to cardiopulmonary bypass. Taking into account lewej z workiem osierdziowym, która stanowiła potencjalną
the patient s past medical history the pericardial defect could przyczynę odczynu osierdziowego na przebyte infekcje dróg od-
be responsible for pericardial adhesions as a reaction to re- dechowych.
current pulmonary infections spreading via persistent pleuro- Słowa kluczowe: wrodzony ubytek osierdzia, ubytek międzyko-
pericardial communication. morowy typu Gerbode, wrodzone wady serca, kardiochirurgia
Key words: congenital pericardial defect, Gerbode septal de- dziecięca.
fect, congenital heart defects, paediatric cardiac surgery.
Background knowledge, we present the first case of a patient with con-
genital pericardial sac defect with congenital Gerbode type
Congenital pericardial defects are rare malformations
ventricular septal defect in an infant described in the lit-
with variable clinical presentations [1, 2]. Pericardial sac de-
erature. The case presented is twice as interesting because
fects can be partial or complete, as it is commonly known in
of preoperative diagnosis of Gerbode defect and necessary
some mammals. The anomalies are still poorly known, the
change of the operative strategy due to destabilizing pres-
majority of literature reports are based on incidentally di-
sure pneumothorax before cardiopulmonary bypass was
agnosed cases, thus it is impossible to ascertain their total
commenced. Taking into account the patient s past medi-
prevalence. Asymptomatic patients remain undiagnosed,
cal history the pericardial defect could be responsible for
otherwise the defects are discovered incidentally during
pericardial adhesions as a reaction to recurrent pulmonary
cardiac surgery. The patients with congenital pericardial
defects are referred for surgery for unrelated conditions, or infections spreading via persistent pleuro-pericardial com-
the diagnosis is given postmortem [1]. To the best of our munication.
Address for correspondence: dr n. med. Ireneusz Haponiuk, Oddział Kardiochirurgii Dziecięcej Pomorskiego Centrum Traumatologii im. Miko-
łaja Kopernika w Gdańsku, ul. Nowe Ogrody 1-6, 80-803 Gdańsk, Polska, tel./fax +48 58 322 08 51, Email: ireneusz_haponiuk@poczta.onet.pl
276 Kardiochirurgia i Torakochirurgia Polska 2010; 7 (3)
WADY WRODZONE
Case report atrium (LV-RA) communication and mild tricuspid regurgita-
tion, the LV function and diameters of the heart chambers
An eight-month-old male infant; 7600 g birth weight,
were normal (Fig. 2).
was admitted to the Department of Paediatric Cardiac
The patient was referred for scheduled ventricular sep-
Surgery, Mikołaj Kopernik Hospital in Gdańsk (Poland)
tal defect repair. After classic median sternotomy and small
with the diagnosis of ventricular septal defect (VSD) and
thymus removal during the pericardiotomy unexpected
hypothyreosis. The boy had a history of permanent respi-
pericardial adhesions in the area of ventricle body and
ratory tract infections prior to the admission with a re-
right atrium (RA) and its appendage (RAA) were found. At
markably bad clinical course. On admission his cardiac
the time of meticulous dissection there was growing left
examination was remarkable for medial displacement of
pressure pneumothorax with hemodynamic destabiliza-
the apex right to the midclavicular line. Preoperative chest
tion, bradycardia and pressure drop. The pneumothorax
X-ray showed flattening and elongation of the left ventricu-
was relieved after insertion of an external suction line into
lar contour and right atrium enlargement (Fig. 1). ECG proved
the left pleura via the pericardial defect, further identified
regular sinus rhythm with the right axis and partial right
in the area of the left atrial appendage (LAA). The pericar-
bundle branch block (PRBBB). Preoperative Echo examina-
dial sac defect was functionally closed by the LAA causing
tion showed 5.4 mm Gerbode VSD with left ventricle-right
air-trapping mechanism of the left pressure pneumothorax.
The heart rotation (40 degrees) in the longitudinal heart
axis was found with the left ventricle (LV) in the front and RA
body situated deep in the pericardium and dorsally. The left
pleura was opened with pleural adhesions as the remnants
of recurrent pleural infections. The cardiac procedure was
performed in cardiopulmonary bypass and moderate hypo-
thermia with antegrade cardioplegic arrest, the heart was
opened through RA incision. The Gerbode defect in the area
of the tricuspid valve annulus with LV-RA communication
was closed with running suture fresh autologous pericardial
patch. The tricuspid valve inspection showed no dysfunc-
tion. The pericardial defect and pericardiotomy were left
open. Postoperative course was uncomplicated. In the first
postoperative echo after surgery there was a small LV-RA
leakage that disappeared in further examinations. The child
was discharged home on the 8th postoperative day in a good
general condition. One year follow-up was uneventful.
Discussion
Congenital pericardial defects are rare findings with
variable clinical presentation and can be complete or partial
Fig. 1. Preoperative chest X-ray showed flattening and elongation
[1, 2]. These are still poorly known anomalies, sometimes
of the left ventricular contour and right atrium enlargement, with
no symptoms that could indicate the presence of left-sided peri- overlooked, with a total number of less than 150 cases re-
cardial sac defects ported. Most frequently pericardial defects are left-sided
(86%) and are related to premature atrophy of the left duct
of Curvier during embryological development. Congeni-
tal complete pericardial absence is thought to be due to
premature atrophy of the left common cardiac vein with
insufficient blood supply to the pleuropericardium, which
leads to its agenesis during pregnancy [1]. Pericardial sac
defects appear three times more frequently in males than in
females in a white-Caucasian population. Thirty percent of
patients demonstrate associated congenital heart defects
as the most frequent ones: atrial septal defect, bicuspid
aortic valve, patent ductus arteriosus, tetralogy of Fallot, as
well as others, like pulmonary sequestration, bronchogenic
cysts, VATER syndrome (vertebral defects, anal atresia, tra-
cheoesophageal fistula, radial and renal dysplasia), Marfan s
syndrome, Pallister-Killian syndrome [1, 3-5]. Pericardial de-
Fig. 2. Preoperative Echo examination showed 5.4 mm Gerbode
fects are more frequent in patients with congenital skeletal
VSD (marked on the scan) with LV-RA communication and mild
tricuspid regurgitation malformations (Holt-Oram syndrome) [2]. Complications
Kardiochirurgia i Torakochirurgia Polska 2010; 7 (3) 277
Congenital pericardial defect with Gerbode type septal defect in rotated heart: a report of a case
associated with pericardial defects depend on their extent. ate an extent of the defect, which provides important infor-
Complete absence of the entire pericardium or absence of mation for further patient s management strategy [1, 9].
the whole left or right side is usually associated with an Therapeutic options are based on small, retrospective
excellent prognosis [6]. A small increase in preload may series which recommend surgical interventions only for pa-
cause ventricular dilatation because of the loss of ventricu- tients with complications related to pericardial deficiency
lar restraint, as it can be demonstrated in volume overload [1, 6]. These include both surgical closure or elongation of
patients with pericardium left open after regular cardiac the pericardial defect with regard to initial problem caused
procedures. One case of severe tricuspid regurgitation due by the pathology [2]. Asymptomatic left total defects usu-
to cardiac hypermobility was reported and it required surgi- ally do not require surgical treatment because of the small
cal intervention [6]. The partial absence is more dangerous. risk of circulatory complications, while the left-sided partial
Entrapment of parts of the heart through defects may lead pericardial defects are more controversial. Small and mod-
to strangulation of the atria, appendages and parts of the erate in size left-sided pericardial defects are considered for
ventricles [7]. Congenital pericardial defect was reported
prophylactic surgery by some, while others suggest treat-
with the presence of acute myocardial necrosis in an adult
ing only symptomatic patients [2, 6, 8]. Otherwise there are
patient free of coronary artery disease, or there was evi- many reports suggesting that both symptomatic and non-
dence of impingement by a pericardial rim [8].
symptomatic patients should be followed by prophylactic
operation to reduce the risk of death from cardiac structure
Clinical presentation of pericardial deficiency is non-spe- herniation and incarceration. On the other hand, patients
cific. In most cases the defect is discovered incidentally in an
with partial absence of the pericardium (isolated); nowa-
asymptomatic patient [2]. There are reports of non-specific
days the focus is to manage the symptoms rather than pro-
symptoms like mild cough, upper respiratory infection symp- phylactic management. An interesting and still more and
toms, brief chest wall throw during exercise usually not as- more available option is thoracoscopy intervention that
sociated with chest pain, shortness of breath, palpitations,
provides safe and effective minimally invasive surgical in-
dyspnoea or respiratory distress leading to syncope [1, 8].
tervention in the area of the pericardium as well as in the
Sometimes the dyspnoea causes the shifting heart symp- heart structures, possible even in a small patient [10].
tom, which is reported by selected patients. Physical exami- In the presented case, we were challenged by the unique
nation may reveal significantly displaced apical pulse area,
situation of a patient with Gerbode defect in a rotated heart
which may be palpated in the anterior or midaxillary line,
and partial left pericardial defect, which was difficult to
as well as basal ejection murmurs, apical midsystolic clicks,
diagnose before surgery because of atypical position of the
or even systolic murmurs of undetermined origin. Patients
heart. Left-sided pericardial sac defects are usually seen
may incidentally present some complications clearly related
with left cardiac border invagination to left pleural space
to the defect, like herniation and incarceration of the myo-
in routine chest X-ray, although the heart borders were
cardium, predominantly the LAA and ventricles. All complica-
changed because of its irregular position. We also consid-
tions have been associated with presentations varying from
ered that both facts (partial absence of left pericardium and
chest pain to infarction, syncope, tricuspid regurgitation
rightward heart axis change) might have not been related
and sudden death [6-8]. The ECG is not pathognomonic and
in embryonic development, thus we cannot exclude two
typically reveals bradycardia and right bundle branch block
independent factors causing right deviation and left-sided
(RBBB), poor R-wave progression secondary to leftward dis-
pericardial defect early in fetal life. Unexpected intraopera-
placement of precordial transitional zone is common, promi-
tive complication made us modify the dissection technique
nent P-waves in the mid-precordial leads denote right atrial
nearby ECC. This complication gives another important
overload. Echocardiographic findings are related to cardiac
argument in the discussion whether pericardial defect can
levoposition and increased mobility within the chest [8].
be of special importance in selected cases.
These include unusual echocardiographic windows marked
change in cardiac position in the direction of the pericardial
hole with changing patient position on the examination ta- References
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