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Congenital Heart Disease in the Adult: What Should the Adult Cardiologist Know?

  • Echocardiography (JM Gardin, Section Editor)
  • Published:
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Abstract

There is an increasing population of adults with congenital heart disease (CHD) due to successful pediatric medical and surgical intervention, and commensurate with this increase is a rise in heart failure, hospital admissions, and hospital costs among adult CHD patients. This group of patients requires careful long-term evaluation and follow-up of the residua and sequelae of their cardiac anomalies that arise in adulthood to prevent late complications. This article addresses congenital heart defects that are encountered in a general adult cardiology practice and reviews clinical, anatomic, and imaging features of each lesion, fundamental management issues, indications for interventions (and often re-interventions), issues related to endocarditis prophylaxis, pregnancy, and appropriateness of referral to a dedicated adult CHD program for long-term care.

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Abbreviations

2D:

Two-dimensional

3D:

Three-dimensional

ASD:

Atrial septal defect

AVSD:

Atrioventricular septal defects

BAV:

Bicuspid aortic valve

CCTGA:

Congenitally corrected transposition of the great arteries

CHD:

Congenital heart disease

CMR:

Cardiac magnetic resonance imaging

CW:

Continuous wave

DCRV:

Double chamber right ventricle

D-TGA:

D-transposition of the great arteries

IVC:

Inferior vena cava

LV:

Left ventricle

PA:

Pulmonary artery

PDA:

Patent ductus arteriosus

PFO:

Patent foramen ovale

PR:

Pulmonic regurgitation

PS:

Pulmonic stenosis

PV:

Pulmonic valve

PW:

Pulsed wave

RV:

Right ventricle

RVOT:

Right ventricular outflow tract

SVC:

Superior vena cava

TOF:

Tetralogy of Fallot

TR:

Tricuspid regurgitation

VSD:

Ventricular septal defect

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Conflict of Interest

Doreen DeFaria Yeh and Mary Etta King declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Author information

Authors and Affiliations

  1. Division of Cardiology, Adult Congenital Heart Disease Program, Echocardiography Section, Massachusetts General Hospital, 55 Fruit St, Yawkey 5-700, Boston, MA, 02114, USA

    Doreen DeFaria Yeh

  2. Division of Pediatric Cardiology, Cardiac Ultrasound Laboratory, Massachusetts General Hospital for Children, 55 Fruit St. Yawkey 5-062, Boston, MA, 02114, USA

    Mary Etta King

Authors
  1. Doreen DeFaria Yeh
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  2. Mary Etta King
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Corresponding author

Correspondence to Doreen DeFaria Yeh.

Additional information

This article is part of the Topical Collection on Echocardiography

Electronic supplementary material

Below is the link to the electronic supplementary material.

AVI video of parasternal short-axis view of young adult with a unicuspid AV. Note the “keyhole” appearance with an open commissure between the non and left coronary cusps, and two raphe. (AVI 15433 kb)

AVI video of the apical four-chamber view in a patient with Ebstein’s anomaly of the tricuspid valve. The left image shows the tethering and apical displacement of the tricuspid valve leaflets with dilated right atrium and atrialized RV. The color Doppler image on the right shows severe TR. (AVI 26911 kb)

AVI video of the apical four-chamber view in a patient with tricuspid atresia post fenestrated lateral tunnel Fontan palliation. At the tricuspid annulus is a fibrous band of tissue, and the right ventricle is hypoplastic. The Fontan pathway through the atrium is seen as a circular structure within the right atrium. (AVI 1242 kb)

AVI video of the color Doppler apical four-chamber view in the same patient showing the flow through the fenestration in the atrial baffle as a small jet entering the common atrium. (AVI 1816 kb)

Appendices

Appendix 1. Echocardiographic Essentials for Assessment of Left and Right Heart Obstruction

Bicuspid Aortic Valve:

  1. 1.

    Morphologic aortic valve features, location of commissural fusion and opening

  2. 2.

    Degree of stenosis and regurgitation

  3. 3.

    Estimated aortic valve area

  4. 4.

    Dimensions of the aortic annulus, sinuses of Valsalva, and ascending aorta

  5. 5.

    LV size, mass, and function

  6. 6.

    Associated left sided obstructive lesions including coarctation, subaortic stenosis, and supramitral ring, additionally ASDs, VSDs and mitral abnormalities

Discrete Subaortic Stenosis:

  1. 1.

    The nature and location of the membrane

  2. 2.

    The degree of LVOT obstruction and aortic valvular regurgitation

  3. 3.

    Aortic annular dimension and aortic valve morphology

  4. 4.

    LV size, mass, and function.

  5. 5.

    Associated defects such as membranous VSD and DCRV

Aortic Coarctation (native or recurrent coarctation):

  1. 1.

    Aortic annulus, sinuses, ascending aorta, transverse arch, isthmus, and descending thoracic aortic dimensions

  2. 2.

    Brachiocephalic branch anatomy

  3. 3.

    Pulsed wave (PW), continuous wave (CW) and color Doppler sampling of the transverse and descending thoracic aorta

  4. 4.

    Pulsed Doppler sampling of the abdominal aorta

  5. 5.

    LV size, mass, and function

  6. 6.

    Aortic valve anatomy and function

  7. 7.

    Associated lesions such as VSD, subaortic membrane, BAV and mitral valve anomalies (mitral prolapse, parachute mitral valve, supramitral ring, aberrant papillary muscle arrangement), aortic arch abnormalities and aberrant subclavian arteries

Valvular PS:

  1. 1.

    Pulmonary valve leaflet morphology and thickness

  2. 2.

    Annular, main PA, and branch dimensions

  3. 3.

    CW Doppler peak and mean gradient

  4. 4.

    Degree of regurgitation

  5. 5.

    Subvalvular hypertrophy or obstruction

  6. 6.

    RV size, wall thickness, and function

  7. 7.

    Doppler TR peak velocity

  8. 8.

    Associated ASD, PFO, VSD, other RV outflow obstruction

Infundibular PS:

  1. 1.

    2D imaging of the mid and distal RV outflow tract in parasternal and subcostal views

  2. 2.

    Color and PW/CW Doppler sampling of the RV outflow tract proximal to the pulmonic valve

  3. 3.

    Pulmonary valve morphology

  4. 4.

    Doppler TR peak velocity

Appendix 2. Echocardiographic Essentials for Evaluation of Congenital Shunt Lesions

Atrial septal defects (native or preprocedural imaging):

  1. 1.

    ASD size and location from multiple windows

  2. 2.

    Septal rims

  3. 3.

    RV size and function

  4. 4.

    Estimated RV and PA pressure from TR and PR jet velocity

  5. 5.

    Associated lesions—PS, mitral valve prolapse, cleft mitral valve, anomalous pulmonary veins, persistent left SVC

Postoperative ASD assessment:

  1. 1.

    Residual shunting

  2. 2.

    Residual pulmonary hypertension

  3. 3.

    RV enlargement and dysfunction

  4. 4.

    RV or RA thrombus

  5. 5.

    Mitral regurgitation from cleft MV or mitral valve prolapse

  6. 6.

    SVC or pulmonary vein stenosis after sinus venosus ASD repair

Post device closure ASD assessment:

  1. 1.

    Device position

  2. 2.

    Residual shunting

  3. 3.

    Device impingement upon systemic or pulmonary venous inflow

  4. 4.

    Device impairment of aortic, mitral or tricuspid valve function

  5. 5.

    Device impingement upon posterior aortic wall

  6. 6.

    Thrombus or vegetation on right or left atrial device facets

Native Ventricular Septal Defect:

  1. 1.

    Size and location of VSD

  2. 2.

    Direction of shunt flow; LV to RV shunting, LV to RA shunting

  3. 3.

    LA size

  4. 4.

    LV size, mass, and function

  5. 5.

    VSD gradient by CW Doppler

  6. 6.

    Estimated RV and PA pressure from TR and PR velocities

  7. 7.

    Presence and degree of RVOT obstruction—DCRV, infundibular, valvar, or branch PS

  8. 8.

    Associated lesions—membranous septal aneurysm, aortic valve prolapse, or discrete subaortic membrane

VSD: Following surgical repair:

  1. 1.

    Residual VSD shunt

  2. 2.

    Residual pulmonary hypertension

  3. 3.

    LV dysfunction

  4. 4.

    TR due to surgical distortion of the septal leaflet

  5. 5.

    Aortic valve prolapse with aortic insufficiency

  6. 6.

    Aortic valve distortion and dysfunction due to VSD patch placement

  7. 7.

    Discrete subaortic membrane and DCRV

Unrepaired Atrioventricular Septal Defect:

  1. 1.

    Size and location of atrial and ventricular defects

  2. 2.

    Presence, direction, and size of ASD and VSD shunt

  3. 3.

    Estimate of RV and PA pressure from TR and PR velocities

  4. 4.

    Specifics of atrioventricular valve anatomy—common AV valve, septal attachments of anterior and posterior bridging leaflets (Rastelli Type A, B, or C); unbalanced alignment

  5. 5.

    Degree of left or right AV valve regurgitation

  6. 6.

    Papillary muscle anatomy and chordal attachments

  7. 7.

    LV size, mass, and function

  8. 8.

    RV size, wall thickness, and function

  9. 9.

    Associated defects—PDA, coarctation, subaortic obstruction

Postoperative AVSD:

  1. 1.

    Residual atrial or ventricular shunt; possible LV-right atrial shunting

  2. 2.

    Residual pulmonary hypertension

  3. 3.

    AV valve regurgitation

  4. 4.

    Discrete subaortic stenosis

  5. 5.

    Assessment of other co-existing preoperative issues

PDA pre- and postintervention:

  1. 1.

    Size and shape of the ductal channel

  2. 2.

    Size and direction of shunt,

  3. 3.

    Aortopulmonary gradient from peak systolic PDA jet velocity

  4. 4.

    LA and LV chamber size; LV function

  5. 5.

    Main PA and branch PA dimensions

  6. 6.

    Aortic arch and isthmus anatomy

  7. 7.

    Device or coil position with specific attention to residual shunt, branch PA stenosis or aortic protrusion

Appendix 3. Echocardiographic Essentials for Assessment of Complex Congenital Heart Disease

Unoperated patient with TOF:

  1. 1.

    VSD location, size and shunt direction; additional VSDs

  2. 2.

    Degree and nature of RV outflow tract obstruction

  3. 3.

    RVOT and branch PA gradients

  4. 4.

    Pulmonary annulus, main and branch PA dimensions

  5. 5.

    RV size, wall thickness, and quantitative assessment of function

  6. 6.

    Coronary artery origins and course

  7. 7.

    Atrial septal anatomy

  8. 8.

    Aortic arch situs

  9. 9.

    Aortopulmonary collaterals

Postoperative patient with TOF:

  1. 1.

    Residual VSD

  2. 2.

    Residual infundibular, valvular or branch PS; DCRV

  3. 3.

    Degree of PR (CW across RV outflow tract)

  4. 4.

    Residual atrial shunting

  5. 5.

    RV volumes, quantitative systolic, and diastolic function

  6. 6.

    Estimated RV pressure from Doppler peak TR jet velocity

  7. 7.

    Aortic valve anatomy and function

  8. 8.

    Aortic root dimensions

  9. 9.

    LV size, global and segmental function

  10. 10.

    Conduit location, size, and gradients, if present

  11. 11.

    Prosthetic pulmonic valve (PV) stenosis or regurgitation, if present

D-Transposition of the Great Arteries after Mustard or Senning procedure (atrial switch):

  1. 1.

    Systemic RV size and function

  2. 2.

    Subaortic and aortic stenosis

  3. 3.

    Subpulmonary LVOT obstruction

  4. 4.

    Estimated subpulmonary ventricular pressure from mitral valve regurgitant velocity

  5. 5.

    Tricuspid valve (systemic AV valve) regurgitation

  6. 6.

    Baffle pathway obstruction

  7. 7.

    Baffle leak (consider agitated saline study if leak not obviously present by Doppler)

  8. 8.

    Associated lesions—VSD, valvar or branch PS, PDA, coarctation

Postarterial switch operation for D-TGA:

  1. 1.

    Aortic root, supravalvar aortic dimensions, and gradient

  2. 2.

    Neo-aortic valve regurgitation

  3. 3.

    Neo-pulmonic valve function

  4. 4.

    Suprapulmonary stenosis in MPA or branches

  5. 5.

    LV size, mass, global and segmental function

  6. 6.

    RV size, wall thickness and function

  7. 7.

    Proximal coronary artery patency

  8. 8.

    Associated lesions—VSD, coarctation

Congenitally Corrected Transposition of the Great Arteries (CCTGA):

  1. 1.

    Identification of visceroatrial situs

  2. 2.

    Establishment of cardiac position—levo-, meso-, or dextrocardia

  3. 3.

    Demonstration of atrioventricular and ventriculoarterial discordance (RA-LV-PA; LA-RV-Aorta)

  4. 4.

    Location, size, and direction of VSD shunt if present

  5. 5.

    Nature and degree of PS or subpulmonary outflow obstruction

  6. 6.

    Anatomy and function of tricuspid valve (systemic AV valve)

  7. 7.

    Size and function of the RV (systemic ventricle)

  8. 8.

    Coronary artery anatomy

Postoperative patient with CCTGA:

  1. 1.

    Biventricular size and function

  2. 2.

    Residual VSD

  3. 3.

    Residual RV or LV outflow obstruction

  4. 4.

    Degree of TR

  5. 5.

    Atrial baffles if present

  6. 6.

    Great artery anastomoses if present

  7. 7.

    Conduit function if present

  8. 8.

    Neo-aortic size and insufficiency

  9. 9.

    Coronary artery patency if translocated

Ebstein’s anomaly:

  1. 1.

    Tricuspid leaflet origin, position, size and tethering

  2. 2.

    Degree of TR

  3. 3.

    RA and atrialized RV size and function

  4. 4.

    True RV size and function

  5. 5.

    Pulmonary valve stenosis

  6. 6.

    Main and branch PA dimensions

  7. 7.

    Atrial septal communication and shunt direction

  8. 8.

    LV size and function

Postoperative Ebstein’s anomaly:

  1. 1.

    Anatomic assessment of tricuspid valve repair or replacement

  2. 2.

    Degree of TR, presence of paravalvular leaking

  3. 3.

    RA size

  4. 4.

    RV size and function

  5. 5.

    LV size and function

  6. 6.

    Patency of Glenn shunt if present

  7. 7.

    Patency of Fontan pathway if present

  8. 8.

    ASD device position and residual shunt if present

Complex CHD: must be individualized to the specific lesion but includes the following basic features:

  1. 1.

    Visceroatrial situs—solitus, inversus, indeterminate

  2. 2.

    Cardiac position—levocardia, mesocardia, dextrocardia

  3. 3.

    Number of atrial chambers and morphology

  4. 4.

    Number of ventricular chambers and morphology

  5. 5.

    Atrioventricular relationship

  6. 6.

    Number of great arteries and their relationship

  7. 7.

    Ventriculoarterial relationship

  8. 8.

    Intracardiac shunts

  9. 9.

    Outflow tract obstruction

  10. 10.

    Other associated anomalies—coarctation, branch PS, systemic and pulmonary venous abnormalities

Complex CHD following the Fontan procedure:

  1. 1.

    Inferior vena cava (IVC) and SVC size and flow pattern

  2. 2.

    Right atrial size

  3. 3.

    Right atrial to PA anastomosis patency and flow patterns, if present

  4. 4.

    Interatrial or extracardiac venous conduit flow patterns

  5. 5.

    Branch PA size and flow pattern

  6. 6.

    Thrombus within the systemic venous pathway

  7. 7.

    Baffle fenestration flow and Doppler gradient

  8. 8.

    Pulmonary venous inflow and left atrial size

  9. 9.

    Atrioventricular valve function

  10. 10.

    Systemic ventricular size and function

  11. 11.

    Outflow tract obstruction

  12. 12.

    Aortic valve stenosis or incompetence

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DeFaria Yeh, D., King, M.E. Congenital Heart Disease in the Adult: What Should the Adult Cardiologist Know?. Curr Cardiol Rep 17, 25 (2015). https://doi.org/10.1007/s11886-015-0579-7

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  • DOI: https://doi.org/10.1007/s11886-015-0579-7

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