Page 19 - Journal of Structural Heart Disease Volume 5, Issue 4

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Meeting Abstracts
Discussion: The overall prevalence of congenital heart disease (CHD) ranges from between 4 to 10 per 1,000 live birth. Several authors have reported the accurate prenatal diagnosis of CHD to range from 27%-36% with increasing detection rates in recent years.
Isolated congenital coronary fistula (CAF) occurs in 1:50,000 live births. Due to its low incidence and the diffi- culty in evaluation of the fetal coronary circulation, prena- tal detection rates are low. There are only 8 reported cases of isolated coronary fistulae diagnosed prenatally.
The prenatal imaging in this patient clearly identified tur- bulent flow at the base of the left atrium. This turbulent flow and the dilated chamber proximal to it (the coro- nary system) were consistent with obstructed pulmonary venous return to either the coronary sinus or to the hepatic venous system. However, the reversal of flow in the aorta indicated the presence of significant runoff as would be expected from a large coronary fistula.
Although most patients with CAF are asymptomatic at diagnosis, heart failure, endocarditis, arrhythmia, and rup- ture of the fistula can be presenting findings. Additionally, premature arteriosclerotic changes within the fistula and thromboembolic events have been described as possible complications. Closure of hemodynamically significant fistulae is recommended. Transcatheter approaches have become increasingly favored as in our case.
14. UNIQUE CAUSE OF SYNCOPE IN A 29-YEAR OLD WOMAN WITH HISTORY OF VENTRICULAR SEPTAL DEFECT AND COARCTATION
Dana Konecny, David Laughrun, Tomas Konecny
University of Southern California, Los Angeles, USA
Case Presentation: 29-year old woman with history of ventricular septal defect (VSD) and aortic coarctation repair was referred for repetitive syncope. Syncopal spells lasted several minutes, occurred yearly, and they coincided with a prodrome of nausea and vomiting. On examina- tion she had no signs of heart failure, no murmurs, equal pulses in both arms, and normal carotid upstrokes without bruits. ECG showed sinus rhythm with bifascicular block but normal PR interval and mild prolongation of QTc to 471. Echocardiogram showed normal heart function with- out detectable VSD. Holter monitor recorded sinus rhythm with a 4-beat run of supraventricular tachycardia. MRA con- firmed normal aortic flow without stenosis or dilatation.
Diagnosis And Treatment: Our differential diagnosis included vasovagal syncope (nausea during prodrome),
infrahisian heart block (existing bifascicular block), ven- tricular tachy-arrhythmia (re-entry around the VSD scar), subclavian steal (left subclavian artery taken down during coarctation repair), supraventricular tachy-arrhythmia (short run on Holter monitor). Implantable loop recorder was placed parasternally in left 6th intercostal space, and after6monthsitsuccessfullyrecordedasyncopalepisode, showing complete heart block with 20 second ventricular asystole. Dual chamber permanent pacemaker was placed with minimum radiation exposure and extra-shielding (patient was at that time in the second trimester of preg- nancy). Syncopal episodes have not recurred during the follow up period.
Discussion: Patients with history of VSD and coarctation repair present unique considerations when evaluating syncope. Their initial coarctation repair may lead to aortic flow impairments with subsequent aneurysmal dilatation or stenosis, but in this case MRA with gadolinium contrast excluded this possibility. Subclavian steal due to missing left subclavian artery (which was taken down for flap repair of the aortic coarctation) can be another potential etiology for syncope via reduction in vertebral artery flow. Patients with hemodynamically significant VSD can potentially suf- fer from left-to-right shunting and resultant Eisenmenger syndrome, but the Echocardiogram excluded significant residual septal defect. The VSD repair in infancy can lead to uniquely delayed rhythm abnormalities: ventricular tachy- cardia due to conduction heterogeneity around the pre- vious VSD, and bradyarrhythmias due to infrahisian block. Ultimately, adult congenital heart disease patients can suf- fer from etiologies common to all patients with syncope: supraventricular tachycardias, vasovagal syncope, ortho- static hypotension, and multiple non-cardiac causes.
Conclusion: Adult patients with unexplained syncope who underwent VSD and coarctation repair in infancy should be evaluated at a specialized center for delayed, potentially lethal consequences of their condition. If imag- ing, hemodynamic, and short-term ECG monitoring results are reassuring, a pro-active serial surveillance should be taken with implantable rhythm monitoring and imaging studies to allow for timely implementation of potentially life-saving interventions.
15. TECHNICAL ASPECTS OF CLOSURE OF RCA TO RV FISTULA
Pablo Teixeirense, Joao Felipe de Toledo, Luiz Antonio Gubolino, Vanessa Sousa, Wilson Guimaraes
Santa Casa de Misericordia de Limeira Hospital, Limeira, Brazil
Hijazi, Z
22nd Annual PICS/AICS Meeting