AAIM Audio Series

Gordon R Cumming MD, FRCPC, FACC

moderator: Marianne E Cumming MSc, MD

March 5, 2014

Case studies: congenital heart

disease

2

Table of Contents / Agenda

Coarctation of the aorta

Ventricular septal defect

Tetralogy of Fallot

Transposition of the Great Arteries

Select short cases

historical treatment

optimal treatment today

favorable / unfavorable factors

optimal testing/information for underwriting

long-term complications

long-term outcome, challenges with limited data

future considerations

3

Congenital heart disease: considerations

Coarctation of the aorta

repair at age 6 (1979)

operative report not obtained

BMI 32.5 kg/m2, treated BP and lipids

Rx lisinopril, simvastatin

BP: 130's/80's throughout APS

majority measured with left arm or unspecified

No exercise stress test in file

Current cardiac MRI and chest MRA performed

4

Case 1: 41 year old male, life insurance

applicant

5

41 year old male: cardiac MRI

Aortic valve tri-leaflet, minimal focal

thickening anterior mitral valve

Normal caliber ascending, descending

aorta, and pulmonary arteries

6

41 year old male: chest MRA

Ventricular septal defect

1964 – Pulmonary banding

1968 - VSD repair (age 4)

No current cardiac evaluation despite recent

cervical spine surgery

APS: "Heart - normal", BPs <120/<80, BMI 24

Agent:

Applicant:

7

Case 2: 49 year old male, life insurance

applicant

Standard of care, 1960's for large VSD

pulmonary artery banding followed by definitive repair age 4

Patient selection in 1960's

Potential long-term complications:

residual pulmonary artery narrowing, possibly elevated RV

pressures

aortic regurgitation or sub-aortic stenosis

arrhythmias

Ideal current follow-up:

resting EKG, Holter, exercise stress test, echocardiogram

VSD standard of care in 2014:

8

49 year old male, VSD

Case 3: 39 year old male, life insurance

applicant

9

Tetralogy of Fallot (TF) was an incidental finding

hospitalized at Johns Hopkins 1977 (age 3) for severe

burn

patch VSD closure with no outflow patch

EKG: complete RBBB, QRS .13; Holter and exercise

EKG: no ectopy

Echocardiogram:

mild-moderate RV enlargement, RVSP 26

moderate pulmonary and mild-moderate tricuspid

regurgitation

no outflow tract obstruction, aortic root 3.8, small PFO

Tetralogy of Fallot; anatomic details

10

Ventricular septal defect (VSD), large*

Right ventricular (RV) outflow obstruction*

Anterior displacement of aorta (overriding aorta)

Right ventricular hypertrophy (RVH)

RV obstruction:

Subvalvular (infundibular) pulmonary stenosis (PS),

both subvalvular and valvular PS or valvular PS only

R-L shunt severity determined by PS severity;

severe PS, large R-L shunts with severe early cyanosis

vs. mild PS, possibly no cyanosis

TF: interventions

11

Palliative (not repair): Blalock-Taussig, Waterston, Potts shunts

Palliative followed by repair:

Before 1980, repair usually postponed until age 5-6 unless cyanosis

With cyanosis, palliative/ central Gortex shunt followed by repair, subsequent

repair often complicated by anomalies, scarring created by shunt, not always able

to correct

Palliative shunts still may be used in severe cases (hypoplastic PA)

Repair:

VSD patch closure

RV outflow tract obstruction relief

removal excess muscle bundles, possible valvotomy

possible outflow tract patch (potential significant PR)

Ideal timing 4 months (although with only mild PS, may delay)

Surgical mortality currently 2% range, long term favorable but

limited follow-up

complete transposition, arterial switch 1989, age 3

weeks

Exam: I/VI short SEM, EKG normal, no symptoms

Echocardiogram:

No additional testing

12

Case 4: 23 year old female, life insurance

applicant

transposition, atrial switch (Mustard operation)

1986, age 2 years

13

Case 5: 30 year old male, life insurance

applicant

Date of download:

2/7/2014

Copyright © The American College of Cardiology.

All rights reserved.

From: Arrhythmia and Mortality After the Mustard Procedure: A 30-Year Single-Center Experience

J Am Coll Cardiol. 1997;29(1):194-201. doi:10.1016/S0735-1097(96)00424-X

Kaplan-Meier estimates of late survival after hospital discharge after the Mustard procedure. Top and bottom lines indicate 95%

confidence intervals.

Figure Legend:

Bicuspid aortic valve (BAV)

with or without dilatation of the aortic root

imaging: echocardiography and MRI

15

Case 6: 25 year old male, life insurance

applicant

Total anomalous pulmonary venous

return (TAPVR)

Partial anomalous pulmonary venous return

(PAPVR)

Anomalous left coronary arteries

AV canal, repaired Primum ASD Subvalvular aortic

stenosis

Dextrocardia: echo findings

Valvular pulmonary stenosis

Rastelli operation in TGA

16

Short cases