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The DDD Mode Pacemaker Therapy and
Long-term Follow-up in a Case With
Congenitally Corrected Transposition of
Great Arteries
Mehmet Akif DÜZENLİ M.D., Nazif AYGÜL M.D., Meryem Ülkü AYDIN M.D.,
Kurtuluş ÖZDEMİR M.D.
Selcuk University, Meram Faculty Of Medicine, Cardiology Department, Konya, Turkey
ABSTRACT
A 19 year-old male with recent syncope complaint and complete atrioventricular (AV) block was admitted
to our clinic. The patient had a history of congenitally corrected transposition of great arteries and associated ventricular septal defect and first degree AV block at the age two. At seven years of age he was found
to be in complete AV block. As ECG demonstrated that complete AV block still continued with a ventricular escape rhythm at a rate of 40 beats/min, a dual chamber pacemaker was implanted. The patient remained asymptomatic for the next three years, and the values of threshold and impedance of the leads implanted in the morphological left ventricle and the right atrium by endocardial approach remained unchanged at
the end of the third years.
K EYWORDS
Congenitally corrected transposition of great arteries, complete atrioventricular block, DDD mode pacemaker.
Doğuştan Düzeltilmiş Büyük Damar Transpozisyonu
Olan Bir Vakada İki Odacıklı Kalp Pili Tedavisi ve
Uzun Dönem Takibi
ÖZET
On dokuz yaşında erkek hasta bayılma şikayetleri ve atriyoventriküler (AV) tam blok nedeniyle kliniğimize
yatırıldı. İki yaşında doğuştan düzeltilmiş büyük arterlerin transpozisyonu ve bununla ilişkili ventriküler septal defekt ve birinci derece AV blok tanısı konan hastada, 7 yaşında AV tam blok gelişti. Hastaya, EKG’de kalp
hızının 40/dak olması ve A V tam bloğun halen devam etmesi üzerine iki odacıklı kalp pili yerleştirildi. Hasta
3 yıllık izlemde asemptomatik olarak kaldı ve endokardiyal yaklaşımla morfolojik sol ventrikül ve sağ atriyuma yerleştirilen pasif fiksasyon leadlerin eşik ve direnç değerleri 3 yıl sonunda değişmedi.
A NAHTAR K ELİMELER
Doğuştan düzeltilmiş büyük damarların transpozisyonu, atriyoventriküler tam blok, iki odacıklı kalp pili
İLETİŞİM ADRESİ
Dr. Kurtuluş ÖZDEMİR
Selçuk Üniversitesi, Meram Tıp Fakültesi, Kardiyoloji Anabilim Dalı; Konya, TURKEY
The DDD Mode Pacemaker Therapy and Long-term Follow-up in a Case With Congenitally Corrected Transposition of
Great Arteries
Introduction
C
ongenitally corrected transposition of great arteries (c-CTGA) is a rare anomaly and
comprises <1% of all forms of congenital heart
diseases (1). In this anomaly, the right atrium
enters the morphological left ventricle and left
atrium enters the morphological right ventricle.
Most common symptoms in these patients
are heart failure, morphological tricuspid valve
failure and symptoms developed secondarily to
atrioventricular (AV) block. In the treatment of
complete AV block, the implantation of permanent pacemaker by endocardial approach has
recently been preferred over the epicardial approach, which requires thoracotomy (2-5). However, there is not enough data concerning the safety of the passive fixation leads implanted dually in the morphological left ventricle and the
right atrium by endocardial approach.
In this case, we aimed to demonstrate the
long term follow-up of a DDD mode pacemaker
implanted endocardially in a c-CTGA case by
using passive fixation leads.
Case Reports
The case report involves a 19-year old male
who was diagnosed to have c-CTGA and associated ventricular septal defect and first degree atrioventricular block at the age of two. At seven
years of age he was found to be in complete AV
block. Since the patients was asymptomatic although complete AV block with ventricular rate
50 beats/min and narrow QRS complex escape
rhythm were detected in a holter study, he was
followed without implanting a pacemaker. He
remained asymptomatic for the next twelve years and had normal exercise tolerance. At the age
of 19, the patient suffering from syncope attacks
was admitted to our clinic. ECG demonstrated
that complete AV block still continued with a
57
ventricular escape rhythm at a rate of 40 beats/
min (Figure 1). A dual chamber pacemaker was
implanted endocardially by using passive fixation leads (Figure 2,3). The procedure was performed without complications and the device was
programmed to DDD rate 60-100/min. The threshold and impedance of the lead implanted in
the right atrium were measured as 0.25V and
669 Ohm, respectively, and the threshold and the
impedance of the lead implanted in the morphologically left ventricle were measured as 1.25 V
and 644 Ohm, respectively. The patient remained asymptomatic for the next three years, and
threshold and impedance values of atrial lead
did not change (0.25 V, 657 Ohm, respectively).
While the threshold value of the ventricular lead
did not change (1.25V), impedance increased but
remained between normal ranges (751Ohm).
Discussion
Atrioventricular conduction anomalies are
seen commonly in c-CTGA and the conduction system is known to be particularly vulnerable for the development of AV block. Of
the patients, >50% show first degree AV block
and >25% develop complete AV block (6).
There are some problems in the treatment of
complete AV block with permanent pacemaker implanted by endocardial approach in patients with c-CTGA: (1) complications due to
the procedure because of the complex anatomy,
(2) difficulties in implanting lead in the morphological left ventricle, which lacks extensive
trabecular network essential for pacemaker lead stability, (3) lack of enough data available
concerning long term function of lead implanted to into morphological left ventricle and cumulative survival.
The function of leads implanted through endocardial and epicardial approaches or the long term
CİLT 6, SAYI 1, Şubat 2007
58
Türk Aritmi, Pacemaker ve Elektrofizyoloji Dergisi
follow-up lead survival has been studied retrospectively in only one study so far. In this study, MarkEstes et al. have demonstrated that long term follow up data among 40 patients with CTGA complicated with complete AV block show no significant difference in the incidence of malfunction
or cumulative lead survival of endocardial leads
when compared with epicardial leads, although
they implanted only VVI endocardial pacemakers
(7). In patients with CTGA, even if they did not have any significant associated structural cardiac defects, (1) more than one third had congestive heart
failure by the fifth decade. AV synchrony known
to contribute to about 20-30% of cardiac output at
rest may be important in patients with c-CTGA.
Many studies have shown that during exercise an
increase in the pacing rate provided by the DDD
mode increase the cardiac output and the duration
of exercise more than fixed frequency VVI pacing
(8). Thus, DDD mode pacemaker may be an advantage in the patients with c-CTGA.
In literature, the patients with c-CTGA who
were successfully implanted DDD pacemaker
were reported, although there are not so many
(2-5). As mentioned previously, in patients with
c-CTGA, morphological LV into which the pacemaker electrode was implanted does not have enough trabeculation, which may cause lead
dislodgement in both short term and long term.
However, in our literature research we could not
find any data concerning the changes in atrial
and ventricular threshold and impedance values
at long term follow up of these patients. In this
case, passive fixation leads were easily implanted in the morphological left ventricle and the
right atrium by endocardial approach without
any complications. In patients with c- CTGA
who run the risk of developing serious morphological right ventricle failure, DDD mode pacemaker may be first considered because the maintenance of the physiology electrical activity of
the heart can be an advantage.
FİGURE 1
ECG showing the presence of complete atrioventricular block with a ventricular escape rhythm at a rate of 40 beats/min.
CİLT 6, SAYI 1, Şubat 2007
The DDD Mode Pacemaker Therapy and Long-term Follow-up in a Case With Congenitally Corrected Transposition of
Great Arteries
59
FİGURE 2
ECG records of the patient after implanted DDD mode pacemaker.
FİGURE 3
Chest X-ray after endocardial pacemaker (DDD mode)
implantation the pati
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