178 • HJC (Hellenic Journal of Cardiology)

Hellenic J Cardiol 2010; 51: 178-182

Case ReportCase Report

Manuscript received:August 1, 2009;Accepted:January 9, 2010.

Address:Ahmet Al Fagih

Department of Adult Cardiology,Prince Sultan Cardiac Center, Riyadh, Kingdom of Saudi Arabiae-mail: aafagih@yahoo.com

Key words: Congenital heart disease, dextrocardia, cardiac computed tomography.

Feasibility of Cardiac Resynchronization Therapy in a Patient with Complex Congenital Heart Disease and Dextrocardia, Facilitated by Cardiac Computed Tomography and Coronary Sinus VenographyAhmet Al FAgih1, KhAled Al NAjAshi2, KhAled dAgriri2, AbdulmAjeed Al OtAy2, sAleh Ahmed Al ghAmdi2

1Department of Adult Cardiology, 2Department of Pediatric Cardiology, Prince Sultan Cardiac Center, Riyadh, Kingdom of Saudi Arabia

We describe a case with pacemaker implantation for cardiac resynchronization therapy (CRT) in a patient with complex congenital heart disease, facilitated by cardiac computed tomography (CT) and coronary sinus (CS) venography. A 37-year-old male presented with congenitally corrected transposition of the great arter-ies and mesocardia, along with a history of two open heart surgeries (closure of atrial septal defects and a ventricular septal defect, and pulmonary valvectomy at age 7; mechanical tricuspid valve replacement at age 13). He showed symptoms of progressive heart failure (NYHA class III) with significant impairment of the systemic right ventricular function. He also developed permanent atrial fibrillation with a junctional rhythm at a rate of 45 beats per minute. Biventricular pacing without an atrial lead was considered to be the best option available. CRT implantation was facilitated by proper identification of CS anatomy utilizing cardiac CT and CS venography and was performed without any complications. At follow up, a postero-anterior chest X-ray showed the final position of the right-sided ventricular (left ventricular morphology) lead pointing to the apex and the left ventricular lead at the posterolateral aspect of the systemic ventricle (right ventricular morphology).

A dult patients with congenital heart disease represent an expanding po-pulation that is growing at a rate

of 5% per annum.1 Ventricular dysfunc-tion is common in adult congenital heart disease patients who have a systemic right ventricle (RV) and is related to electrome-chanical dyssynchrony.2-4 Over 25% of such individuals ultimately progress to symp-tomatic heart failure, which is occasionally refractory to drug therapy and is associated with substantial morbidity and mortality.4 Therefore, identification of novel therapeu-tic strategies in these patients is of critical importance.

Permanent pacemaker leads may be im-planted either epicardially or transvenously in infants as well as older children.5 Patients with a systemic RV may require conven-tional pacemaker therapy,6 which in the presence of ventricular dysfunction and con- duction disease may further compromise cardiac performance.7,8 Hence, devices ca- pable of combining conventional pacing with modern functions geared towards ame-liorating ventricular function seem to offer an obvious advantage.

Although cardiac resynchronization therapy (CRT) is rapidly emerging as an effective strategy for managing ventricular

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Resynchronization in Complex Congenital Heart Disease

dysfunction and heart failure associated with congeni-tal heart disease, its usage in these patients is limited in terms of patient numbers and follow up.9 Experi-ence in transvenous CRT implantation is limited fur-ther by distorted coronary sinus (CS) anatomy and its tributaries due to previous surgeries and intractable heart failure. However, this route may be feasible for future lead implantation at a greater age.5

In this report, we described the feasibility of trans-venous CRT implantation in a patient with congenitally corrected transposition of the great arteries (ccTGA) and mesocardia, along with a history of two open heart surgeries.

Case presentation

A 37-year-old male presented with ccTGA and meso-cardia, along with a history of two open heart surger-ies. At the age of seven years, he underwent surgical closure of atrial septal defects and a ventricular septal defect, and pulmonary valvectomy. He also underwent mechanical tricuspid valve/systemic valve (TV) replace-ment at the age of 13 years. On follow up he showed symptoms of progressive heart failure with significant impairment of systemic RV (RV morphology) func-

tion. He also developed permanent atrial fibrillation with junctional rhythm at a rate of 45 beats per minute. In view of the slow ventricular response and concomi-tant severe heart failure (NYHA class III) symptoms, biventricular pacing without an atrial lead was consid-ered to be the best option available. It was anticipated that the abnormal CS anatomy would be more difficult in this particular patient, hence cardiac computed to-mographic (CT) angiography was performed as a road to implantation procedure (Figure 1).

CRT was performed as a two-step procedure in a single session. Firstly, CS angiography was performed through the right femoral vein approach using a pre-shaped long sheath (St. Jude Medical, SL3) and oc-clusive balloon (Medtronic, Attain) to plan for proper systemic ventricular lead positioning (Figure 2).10 Occlusive venography showed disturbed CS anatomy with only one suitable posterolateral branch, as well as the incidental finding of a fistula connecting the CS to the left atrium (Figure 2).

Subsequently, two axillary vein accesses were ob-tained, and then the RV lead (Medtronic, Sprint Qua-tro, 6947) was implanted in the right-sided ventricular apex (LV morphology). The LV delivery system (9F safe sheath CS Guide) was introduced into right

LV RV

IVC RA

LA

CS

TV

DAO

LV RV

RAIVC

CSDAo

LA

LV RV

RAIVC

CSDAo

LA

Figure 1. An axial computed tomography angiographic view depicting (A) artificial tricuspid valve (TV), right atrium (RA), inferior vena cava (IVC), small fistula into the left atrium (LA). In the three dimensional reconstruction (B) the coronary sinus is color coded in blue. DAO – descending aorta; LV – left ventricle; RV – right ventricle.

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atrium, then into the CS with the help of a 6F Judkins right catheter and 0.035” terumo guide wire (Figure 3). Another CS venography was obtained through the safe sheath to rule out any dissection and to ensure

that the sheath was in the true lumen (Figure 4). A bipolar 4194 LV pacing lead (Medtronic) was then in-troduced into the target vein without much difficulty. The SL3 with the Attain balloon were pulled back through the femoral vein before the safe sheath was withdrawn under fluoroscopy guidance (Figure 5).

The procedure was then completed without any complications. At follow up, the postero-anterior chest X-ray showed the final position of the right-sided ven-tricular (LV morphology) lead pointing to the apex and the LV lead at the postero-lateral aspect of the systemic ventricle (RV morphology) (Figure 6).

Discussion

Congenitally corrected transposition of the great arter-ies accounts for 0.5% of all cases of congenital heart disease, of which 30% might be associated with me-socardia. Additional anomalies may be present, such as ventricular septal defect in 60-80%, sub-pulmonary ventricle outflow obstruction in 30-50% and TV ab-normalities in 14-56% of patients.11,12 Rhythm distur-bances can develop in up to 2% of this population per year, with or without surgical intervention.13,14 In most of these cases, failure of the sub-aortic ventricle usually appears in the third decade of life, with or without cor-rective or palliative surgeries.15

Although pacemaker implantation is considered

Figure 2. Left anterio-oblique view at 45o during occlusive coro-nary sinus venography shows the fistula (black arrow) and the posterolateral branch (white open arrow), as well as the metallic tricuspid valve (white arrow) and the right-sided ventricular tem-porary pacemaker (black open arrow).

Figure 3. Left anterio-oblique view at 25o during coronary sinus (CS) engagement using a 9F safesheath coronary sinus guide (CSG) (white arrow) with a 6F Judkins right catheter (black arrow) and 0.035” terumo guide wire. (a) Negotiating the CS ostium with the guide wire. (b) Guide wire, Judkins right and the safe sheath coronary sinus guide (CSG) inside the CS (striped arrow).

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the best option for dyssynchrony in ccTGA patients, the optimal pacing mode and pacing site in this group of pa-tients are not well established.16,17 There is also evidence to show worsening of heart failure after conventional dual-chamber pacing with the ventricular lead placed in the sub-pulmonary ventricle in ccTGA patients.18,19

CRT appears to be an attractive option for ccTGA patients. In unselected populations with a systemic RV,

4-9% of patients are potentially eligible for CRT.20 However, deleterious effects of unintentional pacing from the middle cardiac vein have been reported in patients with ccTGA.21 The CS is situated adjacent to the systemic RV and drains into the systemic right atrium in ccTGA patients,22 which makes transve-nous CRT feasible in this group of patients, where it is known to improve acute hemodynamics.23 Because of the heterogeneous venous anatomy, visualization of the CS anatomy before attempting CRT implantation is strongly recommended, using different modalities such as CS venography,20 cardiac CT and magnetic resonance imaging. In addition, two-dimensional strain echocardiography is a reliable tool for detecting RV dysfunction.24

Our case highlights the feasibility of transvenous CRT implantation in a patient with a complex con-genital heart disease, involving a systemic sub-aortic right ventricle, provided the procedure is facilitated by proper identification of coronary sinus anatomy utilizing cardiac CT and CS venography. The pres-ence of mesocardia in some of these cases should not exclude them from such beneficial therapies.

Figure 4. Left anterior-oblique view at 20o, coronary sinus venog-raphy from above using a 9F safesheath coronary sinus guide (CSG) with one suitable posterolateral branch (black arrow).

Figure 5. Left anterio-oblique view at 20o showing lead (Medtron-ic 4194) in the posterolateral position (black arrow) emerging from the safe sheath, which was still in the coronary sinus, and the Attain balloon pulled back from the femoral vein (white arrow).

Figure 6. Postero-anterior chest X-ray with the final position of the right-sided ventricular (left ventricular morphology) lead pointing to the apex (white arrow), and the left ventricular lead at the posterolateral aspect of the systemic ventricle (right ventricu-lar morphology) (black arrow).

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