Congenital Coronary Congenital Coronary Arterial FistulaArterial Fistula

Dr. B.ChaitanyaDr. B.Chaitanya

DM Trainee, CardiologyDM Trainee, Cardiology

DefinitionDefinitionCardiac chambersCardiac chambers

Coronary sinusCoronary sinus

Vena cava/pulmonary veinVena cava/pulmonary vein

Pulmonary trunkPulmonary trunk

““arteriovenous/arterial”arteriovenous/arterial”

HistoryHistoryKrause- described in 1865.Krause- described in 1865.

Abbott- Pathology in 1908.Abbott- Pathology in 1908.

Trevor- first autopsy report in 1912.Trevor- first autopsy report in 1912.

Bjork and Crafoord-1Bjork and Crafoord-1stst report of surgical report of surgical correction.correction.

Reidy-first transcatheter embolization -Reidy-first transcatheter embolization -19831983

Angiographic classificationAngiographic classificationType A –Proximal segment dilated to origin of fistula. Type A –Proximal segment dilated to origin of fistula. Distal end normalDistal end normalType B – Coronary artery dilated over entire length.Type B – Coronary artery dilated over entire length.

CAF CAF Incidence : 1 in 50,000Incidence : 1 in 50,00014% of Cong. coronary artery anomalies 14% of Cong. coronary artery anomalies & 0.2% to 0.4% of all Cong. Cardiac & 0.2% to 0.4% of all Cong. Cardiac anomalies anomalies Acquired – interventional proceduresAcquired – interventional procedures1-2% close spontaneously1-2% close spontaneously

EmbryogenesisEmbryogenesisPrimordial epicardial vessels and Primordial epicardial vessels and intramyocardial sinusoids.intramyocardial sinusoids.

Associated conditionsAssociated conditionsTetralogy of FallotTetralogy of Fallot

ASD ASD

PDAPDA

VSDVSD

C-TGAC-TGA

Physiologic CosequencesPhysiologic CosequencesVolume of bloodVolume of blood

Chamber or vascular bedChamber or vascular bed

Drainage is into the RVOT,PT,LA or LV the hemodynamic burden is Drainage is into the RVOT,PT,LA or LV the hemodynamic burden is borne by the LV alone.borne by the LV alone.

Fistula to LV => analogous to because aortic-to-left ventricular Fistula to LV => analogous to because aortic-to-left ventricular flow through the fistula occurs during diastoleflow through the fistula occurs during diastole

RV, Directly into RA or indirectly through CS (RV + LV volume RV, Directly into RA or indirectly through CS (RV + LV volume overload) overload)

Fistula to LA (LV volume overload)Fistula to LA (LV volume overload)

Myocardial ischaemia : Myocardial ischaemia : low resistance pathway => steallow resistance pathway => steal

HistoryHistoryAsymptomaticAsymptomatic

Male:Female is 1:1Male:Female is 1:1

Coronary angio or EchoCoronary angio or Echo

DyspneaDyspnea

FatigueFatigue

Myocardial ischemiaMyocardial ischemia

HFHF

Sudden deathSudden death

IE IE

Rupture Rupture

ClinicalClinicalPulsePulse

JVPJVP

Precordial impulse : large fistula => hyperdynamic left Precordial impulse : large fistula => hyperdynamic left ventricular impulse because of left ventricular volume ventricular impulse because of left ventricular volume overload in all drainage sites overload in all drainage sites

Fistula => LA or LV or PA, an isolated left ventricular Fistula => LA or LV or PA, an isolated left ventricular impulse impulse

Fistula =>RA or RV, volume overload is imposed on both Fistula =>RA or RV, volume overload is imposed on both ventriclesventricles

AuscultationAuscultationA continuous murmur is an auscultatory hallmark A continuous murmur is an auscultatory hallmark

May be mistaken for a PDAMay be mistaken for a PDA

Distinction => configuration of the murmur & its Distinction => configuration of the murmur & its location (determined by the drainage site) location (determined by the drainage site)

Drainage site Location of murmur

RA directly or indirectly via CS

Rt. upper or lower sternal border or over the sternum

LCX to CS Back between the spine and left scapula

Inflow tract of the RV mid to lower LSB, over the lower sternum or subxiphoid

RVOT Upper to mid LSB

PT Upper LSB

LA upper LSB and may radiate toward the left anterior axiliary line

Coronary arterial fistulas that are Coronary arterial fistulas that are likely to be mistaken for a likely to be mistaken for a PDA PDA are those that drain into the PT, LA or RVOTare those that drain into the PT, LA or RVOT

Because the majority of coronary arterial fistulas drain into Because the majority of coronary arterial fistulas drain into the body of RV or RA, the accompanying continuous the body of RV or RA, the accompanying continuous murmurs are heard at sites remote from the ductus location. murmurs are heard at sites remote from the ductus location.

The configuration of continuous murmurs from these The configuration of continuous murmurs from these different fistulous sources differ from each other and differ different fistulous sources differ from each other and differ from the continuous murmur of PDA. from the continuous murmur of PDA.

Fistula draining into the RA, CS, LA, pressure gradients are Fistula draining into the RA, CS, LA, pressure gradients are larger during systole than during diastole, so the murmur is larger during systole than during diastole, so the murmur is louder in systolelouder in systole

When the fistula communicates with the pulmonary trunk, When the fistula communicates with the pulmonary trunk, the continuous murmur may increase around the second the continuous murmur may increase around the second heart sound as in patent ductus but does not contain eddy heart sound as in patent ductus but does not contain eddy sounds. sounds.

Flow patterns are more complex when the fistula drains into Flow patterns are more complex when the fistula drains into RV because right ventricular contraction compresses the RV because right ventricular contraction compresses the fistula during its fistula during its transmural course transmural course

Compression reduces systolic flow, so the systolic portion of Compression reduces systolic flow, so the systolic portion of the continuous murmur softens. the continuous murmur softens.

If the fistula is only moderately compressed by right If the fistula is only moderately compressed by right ventricular contraction, the intramural systolic gradient ventricular contraction, the intramural systolic gradient increases, so the systolic portion of the continuous murmur increases, so the systolic portion of the continuous murmur increases during systole increases during systole

CHF as RVEDP increases, a continuous murmur may be CHF as RVEDP increases, a continuous murmur may be confined to systole because elevated diastolic pressure confined to systole because elevated diastolic pressure decreases diastolic flow through the fistula.decreases diastolic flow through the fistula.

EDM => fistula drains into LV. EDM => fistula drains into LV.

S2 splits normally during respiration even when the S2 splits normally during respiration even when the right atrium receives the fistula. right atrium receives the fistula.

2 reasons. 2 reasons.

First, the shunt volume is shared equally by the right First, the shunt volume is shared equally by the right and left sides of the heart because shunted blood must and left sides of the heart because shunted blood must flow through both ventricles on its way back to the flow through both ventricles on its way back to the aorta. aorta.

Second, inspiration results in an increase in right Second, inspiration results in an increase in right ventricular stroke volume and a decrease in left ventricular stroke volume and a decrease in left ventricular stroke volume because the shunt into the ventricular stroke volume because the shunt into the right atrium occurs with an intact atrial septumright atrium occurs with an intact atrial septum

LOCATIONLOCATION

ECGECGRt atrial P wave => RA or CSRt atrial P wave => RA or CS

Lt.atrial P wave => RV, PT, LALt.atrial P wave => RV, PT, LA

AF occasionally occurs in older patients with fistulas that AF occasionally occurs in older patients with fistulas that drain into the RA,LA,CSdrain into the RA,LA,CS

LVH => RVOT, PT, LA, LVLVH => RVOT, PT, LA, LV

BiVH => RA or RV (because shunted blood circulates through BiVH => RA or RV (because shunted blood circulates through both ventricles)both ventricles)

Coronary steal => ST segment and T wave changes (s/o Coronary steal => ST segment and T wave changes (s/o ischemia)ischemia)

CXRCXR• Volume and duration of flow and the site of drainage. Volume and duration of flow and the site of drainage. • Young patients with small fistulas have normal x-raysYoung patients with small fistulas have normal x-rays• Infants with large fistulas, CHF =>cardiomegalyInfants with large fistulas, CHF =>cardiomegaly• Large fistula that drains into the RA or CS => increased Large fistula that drains into the RA or CS => increased

pulmonary vascularity, biventricular & biatrial enlargementpulmonary vascularity, biventricular & biatrial enlargement• RV => similar picture but without dilatation of RA. RV => similar picture but without dilatation of RA. • PT or LA => enlargement is confined to LA & LVPT or LA => enlargement is confined to LA & LV• Multiple saccular aneurysms => irregular silhouette along Multiple saccular aneurysms => irregular silhouette along

the right or left cardiac border the right or left cardiac border • Rarely calcification of wall of fistulaRarely calcification of wall of fistula

RCA -RARCA -RA

After AF

ECHOECHO

MRIMRI

CATHCATH

Coronary angiographyCoronary angiography

ManagementManagement

•SurgicalSurgical•Transcatheter closureTranscatheter closure

•Elective closure of significant Elective closure of significant CAVF in childhood.CAVF in childhood.

SURGICALSURGICAL• The goal of treatment is to occlude the fistula whilst The goal of treatment is to occlude the fistula whilst

preserving normal coronary arterial flow. preserving normal coronary arterial flow. • No. of fistulous connections, the nature of the feeding No. of fistulous connections, the nature of the feeding

vessel or vessels, the sites of drainage & vessel or vessels, the sites of drainage & quantification of myocardium at risk for injury or lossquantification of myocardium at risk for injury or loss

• When the lesion is clearly visible, closure can be When the lesion is clearly visible, closure can be achieved, with-out extracorporeal circulation, by achieved, with-out extracorporeal circulation, by clamping and suturing the fistulous channels on the clamping and suturing the fistulous channels on the outside of the heartoutside of the heart

• Cardio-pulmonary bypass => need to enter a chamber Cardio-pulmonary bypass => need to enter a chamber to close the fistula from within, or if insertion of a to close the fistula from within, or if insertion of a bypass graft is necessary to maintain viability of the bypass graft is necessary to maintain viability of the myocardium distal to the ligated artery. myocardium distal to the ligated artery.

Transcatheter closureTranscatheter closure• The aim of closure by catheterisation is to occlude The aim of closure by catheterisation is to occlude

the artery feeding the fistula as distally, and close the artery feeding the fistula as distally, and close to its point of termination, as is possible to avoid to its point of termination, as is possible to avoid occlusion of branches feeding normal myocardium.occlusion of branches feeding normal myocardium.

• Age and size of the patient, the size of the catheter Age and size of the patient, the size of the catheter that can be used in the patient, the size of the that can be used in the patient, the size of the vessel to be occluded, and the tortuosity of the vessel to be occluded, and the tortuosity of the course course

Transcatheter closureTranscatheter closure• Coils- Gianturco Coils- Gianturco

Excellent retrievability

Vascular plugsVascular plugs

*Larger & easily approachable from Rt. side

Double umbrella devices Double umbrella devices CardioSEALCardioSEAL

Amplatzer deviceAmplatzer device

ComplicationsComplications• Inadvertent embolisation of coilsInadvertent embolisation of coils• Transient electrocardiographic T-wave changesTransient electrocardiographic T-wave changes• Transient BBB Transient BBB • Myocardial infarction Myocardial infarction

THANK YOUTHANK YOU