Diagnostic Catheterization in Childhood and Adult Congenital Heart Disease

Diagnostic Catheterization in Childhood and Adult Congenital Heart Disease

Gabriele Egidy Assenza

James E. Lock

Michael J. Landzberg

As a growing population of adults reaches adulthood after being treated during infancy for congenital heart disease,1 a significant proportion of patients undergoing cardiac catheterization for congenital cardiac abnormalities now are older than 18 years of age; a progressive increase in the agedistribution of cardiac congenital catheterization is expected. Data from Boston Children’s Hospital show that, for the period 2005-2011, approximately 20% of the catheterization laboratory case load of this tertiary referral center for treatment of congenital heart disease is composed of adult patients.1 Despite continuing advances in noninvasive anatomic and physiologic imaging modalities, precise catheter-based definition of anatomy and physiology remains invaluable in the primary and adjunctive management of many of these pediatric and older patients with congenital heart disease.2 It can elucidate the following:

Table 9.1 Confounding Physiologic Abnormalities in Patients with Congenital Heart Disease

Abnormalities in atrial or preventricular transport

Alterations in pulmonary blood flow (quantity, pulsatility, resistance)

Variations in pulmonary capacitance (conduit vessels)

Shunt-, obstruction-, or impedance-related changes in ventricular loading conditions

Myocardial-pericardial interaction

Abnormalities of electrical conduction

1) The varying, and sometimes unique, “natural” (unoperated) and post-operated anatomies, and the associated physiologic consequences thereof (Table 9.1).

2) The codependency between the pulmonary (ventricle/vasculature/parenchymal) and the systemic circulation.

3) The nature and precision of catheter-based anatomicphysiologic correlations.

4) Precise angiographic resolution of small (<2 to 3 mm) or tortuous structures, as well as for definition of multiple entry or exit sites or connections.2

5) The increasing patient interactions with premature acquired noncardiovascular disease. In fact, increasing awareness of the neurohormonal and functional limitations in children and adults with congenital heart disease suggest an ever-increasing role for greater physiologic and anatomic understanding of such patients.3

In past decades, the majority of patients with congenital heart disease presented with one of several classes of “simple” anatomy or physiology, including obstructions (pulmonary/aortic stenosis [PS/AS], aortic coarctation [CoA]), or intravascular shunts (atrial/ventricular septal defects [ASD/VSD], patent ductus arteriosus [PDA]). At present, the majority of surviving patients have increasingly complex anatomy and physiology,4 since greater than 60% of adult congenital heart disease patients have had at least one surgery prior to their adult years (and approximately half of these patients have had a reoperation during adulthood). A complete review of every aspect of individual “natural” and prior operated history (Table 9.2) and anatomy (with particular attention to the specifics of each intervention) is required prior to embarking upon any catheter-based investigation (Table 9.3). This should be coupled with a full understanding of potential anatomic and physiologic variations and sequelae, as well as awareness and potential to perform intervention, as needed. As with investigation of patients with acquired heart disease, a detailed preprocedural investigational plan—with attention to adequately trained support staff, available tools, and preprocedural and postprocedural monitoring—is required.

Table 9.2 Typical Categorization of Surgical Repairs


Typical Lesion Application

Surgical Connection

Glenn (Classic)

Single ventricle/TA

SVC to (right) pulmonary artery



Single ventricle/TA



Fontan (atriopulmonary)

Single ventricle/TA

Atrial appendage to RV or PA


IVC-SVC intra- or extracardiac baffle to PA’s


TOF/DORV/pulmonary atresia

Ascending aorta to RPA

Side-to side


TOF/DORV/pulmonary atresia

Descending aorta to LPA


Blalock-Taussig (classic)

TOF/DORV/pulmonary atresia

Subclavian artery to branch PA



TOF/DORV/pulmonary atresia

Conduit from subclavian artery to branch PA


Mustard/Senning (atrial switch)


Baffle directing SVC-IVC flow to subpulmonary LV, pulmonary venous flow to subsystemic RV


Arterial switch


Translocation of moreposterior MPA to anterior supra-LV position, moreanterior aorta to posterior supra-PA position, coronary arterial reimplantation



Conduit between subpulmonary ventricle and PA



Translocation of proximal MPA to supra-LV position, end-to-side anastomosis of distal MPA to aorta, modified Blalock-Taussig shunt

Double switch


Atrial switch + arterial switch

Note: All patients have variations mandating detailed review of operative reporting.

DORV, double outlet right ventricle; HLHS, hypoplastic left heart syndrome; IVC, inferior vena cava; LPA, left pulmonary artery; LV, left ventricle; MPA, main pulmonary artery; PA, pulmonary artery; RPA, right pulmonary artery; RV, right ventricle; SVC, superior vena cava; TA, tricuspid atresia; TGA, transposition of the great arteries (L, left; D, right); TOF, tetralogy of Fallot.

Table 9.3 Typical Indications for Diagnostic Catheterizations/Preferred Imaging Modalities/Interventions

Typical Lesion/s

Diagnostic Cath Typical Indications

Preferred Imaging Modalities

Cath Indication: Interventional

ASD secundum

No: useful for PVR when PHT suspect → ASD test occlusion; PHT vasodilator testing; HD-based management of RV and LV dysfunction


ASD closure




PFO closure when indicated

ASD sinus venosus

Debated: higher incidence PHT: useful for PVR when PHT suspect; see above


ASD primum



AV canal defect

No: with increasing age, increased risk of PHT→ check PVR; see above



Debated: PVR, PV anatomy and rule out stenoses


VSD, membranous

No: uncommon need to assess PVR


Investigational closure

VSD, multiple muscular

No: HD-based management of ventricular dysfunction, when indicated


VSD closure

Ao stenosis/regurgitation: subvalvular/supravalvar

Debated: Hemodynamic changes remain the standard for intervention in children and young adults with valvar AS Supravalvar AS: Useful to define relationship to CA origins
AR: demonstration of fistulous connections when indicated


AS: valve dilations

Aortic coarctation

No: Hemodynamic changes remain the standard for intervention in children and adults




No: PA pressure when PHT suspect → PDA test occlusion


PDA closure

Valvar PS

No: HD-based management of RV failure when appropriate


Valve dilation

Peripheral PS

No: HD-based management of RV failure or PHT when appropriate

Nuclear scintigraphy/MRI

PA dilation/stent

TOF, preoperative

No: Anatomy when CA’s, VSD’s, Ao-PA collaterals cannot be otherwise sufficiently imaged


Close muscular VSD’s

TOF, postoperative

Assess for residual shunts; HD-based management of RV or LV dysfunction; PHT therapy


Close residual shunts/VSD’s; PA or conduit dilation/stent

TOF, pulmonary atresia

Yes: define PA anatomy and hemodynamics


Close Ao-PA connections; dilate/stent stenoses

Pulmonary atresia/intact septum

In children, define coronary anatomy; in adults, define CA anatomy or HD-based management of ventricular dysfunction, as indicated

TGA-D, preoperative



Atrial septostomy

TGA-D, postoperative atrial switch

Assessment of residual shunting; HD-based management of systemic ventricular dysfunction or PHT


Shunt closure

TGA-D VSD/PS;Truncus; DORV postoperative

No; HD-based management of systemic ventricular dysfunction or PHT


Shunt closure; conduit dilation/stent

TGA-D, postoperative arterial switch

Assessment of PA stenoses, coronary arterial stenoses


CA dilation/stent


HD-based management of systemic ventricular dysfunction


Single ventricle, preoperative

Yes: hemodynamics/PVR


Close collaterals, PA dilation/stent

Single ventricle, post-Fontan

Yes: HD-based management of load and ventricular function


Conduit and PA dilation/stent; close collaterals

Ao, aorta; AR, aortic regurgitation; AS, aortic stenosis; ASD, atrial septal defect; AV, atrioventricular; CA, coronary artery; DORV, double outlet right ventricle; HD, hemodynamics; ICE, intracardiac echocardiography; IVUS, intravascular ultrasonography; LV, left ventricle; MRI, magnetic resonance imaging; PA, pulmonary artery; PFO, patent foramen ovale; PHT, pulmonary hypertension; PS, pulmonary stenosis; PV, pulmonary valve; PVR, pulmonary vascular resistance; RV, right ventricle;TAPVR, total anomalous pulmonary venous return; TEE, transesophageal echocardiography; TGA, transposition of the great arteries (L, left; D, right); TOF, tetralogy of Fallot; TTE, transthoracic echocardiography; VSD, ventricular septal defect.


Vascular Access/Vessel and Chamber Entry

Although usual femoral or jugular arterial and venous access can be used in larger children and adults (see Chapter 6), special access routes are usually required in neonates and infants. Options for vascular access vary depending on body habitus, vessel patency, and area to be accessed. Patient size, chamber dilation, and vessel distortion present additional technical challenges that can usually be overcome with experience.

Umbilical Vessels

Umbilical vessels have decreasing patency over the first 72 postnatal hours, but their use allows sparing of other vessels. Vascular access via the umbilical vein (5F umbilical catheter entry) directs catheter position posteriorly in the right atrium, which assists balloon atrial septostomy but adds considerable difficulty to achieving stable right ventricle (RV) and pulmonary artery (PA) access. Given the nearly 180° turns involved in catheter passage (umbilical vein [UV] → portal vein → ductus venosus → inferior vena cava [IVC] → right atrium), concomitant angiographic delineation of course during entry is suggested. Hand-administered contrast injection to demonstrate ductus patency, combined with either use of a tip-deflecting or torque-controlled wire, permits posterior advancement of the catheter, avoidance of intubation of the liver, and successful passage of the UV catheter to the IVC, where it is exchanged for an access sheath after angiographic corroboration. Likewise, the additional curve required to pass a catheter from the umbilical artery (patent for up to 7-10 days post-natally) through the iliac artery may decrease success of retrograde catheter passage to the systemic ventricle, although this maneuver is frequently successful.

Direct Hepatic Vein

Direct hepatic vein entry can be considered when the femoral veins are impassable.5 A Chiba needle is passed between the mid and anterior axillary line, near the costal margin between the diaphragm and the inferior liver edge. The needle is typically advanced using ultrasound guidance, passing posteriorly and cephalad toward the intrahepatic IVC or just caudal to the IVC-right atrial (RA) junction, to within a few centimeters of the right border of the spine. Contrast injection confirms entry into a large central hepatic vein, where a sheath and dilator are advanced over a guidewire to the RA. Large sheath entry and transseptal passage can be performed without complication via this route. At end of procedure, a catheter 1F size smaller than the entry catheter is exchanged, and this sheath is withdrawn, with hand injection of contrast until the sheath is out of the vessel and within the liver parenchymal tract. This tract is then filled with either coils or gelfoam, with subsequent pain at the entry site expected for an ensuing 24 hours, due to peritoneal irritation.

Intracardiac Catheter Manipulation

Even after vessel access, catheter passage into the desired chambers may require particular knowledge and experience. Appropriate catheter positioning may be facilitated by use of torque-controllable, extendible, tip deflecting, stiff, extrastiff, 0.035 inch and 0.038 inch guide wires, as well as by increased use of shaped catheters designed for peripheral or coronary use.

Entry to the superior vena cava (SVC) is easiest via advancement of a straight wire or catheter from the IVC (soft catheters tend to advance anteriorally toward the atrial appendage, away from the SVC). A straight catheter may be gently advanced with a soft counterclockwise rolling to ensure freedom of the catheter tip. Foreshortening of the catheter tip in the anteroposterior (AP) projection typically marks successful posterior angulation, permitting advancement of the free catheter tip to the SVC, avoiding the more anterior right atrial appendage. Particular caution in catheter manipulation is required in those patients with history of atrial arrhythmias or in those patients where a rapid atrial tachycardia could lead to acute hemodynamic decompensation (such as severe pulmonary hypertension, severe left ventricular outflow tract obstruction, mitral valve stenosis, and others).

On occasion, interruption of the IVC, with azygous continuation, may complicate catheter passage, markedly elongating the catheter course. Multiple curves along the catheter course make further posterior or transseptal passage extremely difficult from this access.

Passage to the RV may be complicated when (1) the RA is excessively large; (2) the tricuspid valve (TV) or RV is diminutive; (3) marked TV regurgitation is present; (4) pulmonary atresia is present. Entry can be facilitated by either advancement of a preformed catheter with curvature aimed toward the TV, or with a soft-tipped catheter into which may be introduced the preformed bend on the stiff end of a wire or a tip-deflecting wire, always leaving the wire within the catheter rather than allowing it to protrude into the vasculature. The guidewire-soft-catheter technique allows for adjustment of entry angle and length of curvature by balancing the distance of the guidewire tip from the catheter end, prior to catheter advancement over the guidewire. Particular care must be taken with this approach to ensure that the catheter tip is moving freely, prior to further manipulation or balloon tip inflation.

Intubation of a normally positioned RV outflow/main pulmonary artery (MPA) may be difficult when (1) the RV is particularly dilated, or (2) the TV is regurgitant. Passage via an internal jugular or subclavian vein approach may increase
stability and aid in anterior angulation to and through the RV outflow tract. A multipurpose or similarly precurved softtipped catheter can be turned gently in clockwise fashion, with either concomitant contrast injection or use of a torquecontrolled wire. Similarly, a soft balloon end-hole catheter can be stiffened at its distal end either with a sharp S-shaped bend to the stiff end of a 0.035 inch guidewire wire, or with a tip-deflecting wire, to facilitate passage to the RV outflow tract. An alternative approach requires the creation of a controlled loop in the RA to enhance catheter stability to engage the RV outflow and MPA.

Passage of a catheter from the femoral vein through the RV outflow typically is directed toward a normally positioned, posteriorly directed left pulmonary artery (LPA). When the PA’s are in altered positions, or are dilated, shaping the stiff end of a guidewire with a compound clockwise or counterclockwise loop, and advancing it to the end of a soft catheter may help direct the catheter to the right or to the left PA, respectively. Such compound curves may prove extremely useful in individual circumstances, transforming basic shaped catheters into individualized, “custom-fitted” entry devices. For example, use of a similar, tight, S-shaped compound guidewire curve can assist in directing a catheter from the proximal branch right pulmonary artery (RPA) to the upper lobe vessel. Similarly, a preshaped catheter can be used with contrast injection or a torque-controlled guidewire to assist branch PA entry. Intubation of a dilated or angulated branch RPA may be particularly difficult from a femoral approach, and we have found that retraction of a left Judkins coronary catheter from the LPA into the MPA and angulated toward the right often facilitates RPA entry.

Only gold members can continue reading. Log In or Register to continue

Jun 26, 2016 | Posted by in CARDIOLOGY | Comments Off on Diagnostic Catheterization in Childhood and Adult Congenital Heart Disease

Full access? Get Clinical Tree

Get Clinical Tree app for offline access