Other congenital heart disease in adults

Question 1

Patent ductus arteriosus
Types
Important measurements of the duct

Answer 1

Toronto classification based on the shape of the duct.
Theres types A-E.

Aortic ampula Diameter
Aortic ampula Length
Aortic ampula midpoint diameter
Narrowest Diameter
Pulmonary artery ampula diamter

Question 2

Patent ductus arteriousus
consequences
treatments

Answer 2

Pulmonary hypertension
Dyspnea

Congestive heart failure.

Treatment, Transcatheter closure. Very effective.

Question 3

Tetralogy of Fallot

Answer 3

Overriding Aorta, receiving from both chambers

VSD

Pulmonary stenosis/RV outflow obstruction

Right ventricular hypertrophy

Question 4

Late problems from repaired Tetralogy of Fallot

Answer 4

Pulmonary regurgitation - significant if PR fraction is above 20%,
RV outflow re-stenosis/obstruction
RV dilation and dysfunction - EF <40%
Eventual Tricuspid regurgitation in 30%
RV fibrosis can occur and has bad prognosis, significant arrhythmia SCD chance.

Scars from the ventriculotomy – Foci for arrhythmia

Ventricular arrhythmias in about 50% of patients
V.Tach in 10-15%

RBBB and LAH 10-25%

Complete AV block 2%

Question 5

Problems with the two different kinds of RV outflow obstruction repairs.

Answer 5

Trans-annular patch treatment: Always causes free pulmonary regurgitation and eventual RV volume overload, hypertrophy and pulmonary hypertension.

RV-PulmArtery graft treatment: Used for extreme pulmonary stenosis or atresia of pulmonary artery.
They all eventually need to be replaced:
1- children outgrow them
2- there is progressive lumen obstruction, in both prosthetic or homograft types.
Fibrotic intimal proliferation, graft contraction, external compression, calcification, kinking all contribute.
by 10 years about 50% have had to be replaced.

Question 6

Indications for Pulm Valve Replacement after TOF repair

Answer 6

Severe pulm. regurg, with symptoms or exercise intolerance.

Severe PR and RV dysfunction or severe RV enlargement

Symptomatic or sustained arrhythmias

Development of Tricuspid Regurg.

Question 7

Arrhythmia management after TOF repair

Answer 7

First attempt electrophys mapping, and ablation,
Focus is usually in the RVOT between the patch and the pulmonary annulus.

Implant ICD in patients with symptomatic V.Tach/Fib, high risk of sudden death.

Question 8

Summary of TOF repair late problems.

Answer 8

Residual pulmonary regurgitation
● RV dilation and dysfunction from PR, possibly with
associated TR
● Residual RVOTO
● Branch PA stenosis or hypoplasia
● Sustained VT
● Sudden cardiac death
● AV block, atrial flutter, and/or atrial fibrillation
● Progressive AR

Question 9

Transposition of the great arteries, repair types and consequences

Answer 9

Intra atrial repair, transposing the great veins to match the arteries. RV pumps to aorta and LV to pulmonary.
-Severe late problems.
Tricuspid regurg and RV failure
Sinus node dysfunction and atrial arrhythmia

New surgical methods relocate the aorta and PA to their correct ventricle. Far fewer late consequences.

Question 10

Pulmonary stenosis

Answer 10

Often presents in adult life

Effectively treated with balloon valvuloplasty, which is only done if there is more than 30mmHg gradiaent across the pulm valve.
Very good prognosis. Minimal complications.