Download The Adult Congenital Heart Disease Patient

The Adult Congenital Heart
Disease Patient
Michael Luna, MD
Assistant Professor
Department of Internal Medicine
Interventional Cardiology
Adult Congenital Heart Disease Program
UT Southwestern Medical Center
Disclosures
I receive research funding from AstraZeneca.
Field of Adult Congenital
Heart Disease
• Reparative surgery for congenital cardiac
lesions has lead to dramatic improvement in
survival
• ~85% will survive into adulthood
• In 2000, 32nd Bethesda Conference reported
an estimated 800,000 patients living with
some form of ACHD
Marelli et al. Circulation. 2007;115:163–72.
Warnes et al. J Am Coll Cardiol. 2001;37:1170 –5.
Scope of Congenital Heart
Disease in the Cath Lab
• Diagnostic
catheterizations
– shunt calculation
– pulmonary hypertension
– complex cyanotic
congenital lesions
– angiography
• Interventional
catheterizations
– Shunt lesion occlusions
• device occluders
• coils
• exclusion by covered
stent
– Vascular stenting
• bare metal stents
• covered stents
• drug-eluting stents
– Valve implantation
• Melody valve
27 year old with known
murmur and progressive
exertional dyspnea
• G3P3 with uncomplicated deliveries
• Delivered third child at our institution
• Referred for chest wall echocardiogram
27 year old with known
murmur, progressive exertional
dyspnea
27 year old with ASD (secundum),
VSD (membranous), PDA (type A)
27 year old with ASD (secundum),
VSD (membranous), PDA (type A)
27 year old with ASD (secundum),
VSD (membranous), PDA (type A)
27 year old with ASD (secundum),
VSD (membranous), PDA (type A)
36 year old with PAH
associated with CHD
• Prior professional ballerina
• Severe, progressive exertional dypsnea in late 20s
• Diagnosed with PAH and referred to our Pulmonary
Hypertension group
• Initially felt to be iPAH but sinus venosus defect with
anomalous drainage of RUPV/RMPV was detected by
cath
–
–
–
–
Bidirectional shunting (Qp/Qs = 1.5:1)
Rp ~12 Wood units
Rp decreased to ~6 Wood units with pulmonary vasodilators
Referred for surgical repair of the sinus venosus
defect/PAPVR
36 year old with Sinus
Venosus Defect/PAPVR
36 year old with Sinus
Venosus Defect/PAPVR
Single-patch technique involves baffling of the
anomalous pulmonary veins to the left atrium without
causing SVC obstruction
36 year old with Sinus
Venosus Defect/PAPVR
36 year old with Sinus
Venosus Defect/PAPVR
36 year old with Sinus Venosus
Defect/PAPVR
36 year old with Sinus Venosus
Defect/PAPVR
36 year old with Sinus Venosus
Defect/PAPVR
36 year old, born with
severe valvar pulmonic
stenosis
• Underwent pulmonary valvectomy at 3
years of life
• Surgical PVR ~8 years ago due to
progressive RV dilation 21 mm
Carpentier-Edwards Magna Valve
36 year old, born with
severe valvar pulmonic
stenosis
•
•
•
•
•
BMI ~56
OSA on CPAP
HTN
Hypothyroidism
DVT/PE history
Recurrent Pulmonic Stenosis
Therapeutic Options?
RVOT Dysfunction
• Many congenital
cardiac defects require
surgical RVOT
reconstruction:
– placement of a right
ventricle (RV) to
pulmonary artery (PA)
conduit
– PV valve replacement
with bioprosthesis
RVOT Lesions
• RVOT/PV stenosis/atresia
• Absence of the pulmonary artery
• Pulmonary insufficiency
Pulmonic Valve Bioprosthesis
• Long term performance
remains poor
• Controversy regarding
differential performance
of bioprosthetic material
Jang et al. European Journal of Cardio-Thoracic Surgery 42 (2012) e1–e8.
RV-PA Conduit Outcomes
Multiple surgical
procedures for conduit
replacement or revision
is common
Ong et al. Am J Cardiol. 2013 Jun 1;111(11):1638-43.
Treating Conduit Dysfunction
• Surgical conduit replacement/revision
• Bioprosthetic valve placement
• Percutaneous valve placement
Transcatheter Pulmonary
Valve Replacement
• Allows for pulmonary valve replacement
without cardiopulmonary bypass in
appropriate patients
–
–
–
–
RV-PA conduit 16 mm and larger
Bioprosthetic valves
Anatomy amenable to implant
Adequate patient size (22 Fr system)
• 30 kg
Transcatheter Pulmonary
Valve Replacement
36 year old with repaired
congenital PS
36 year old – Melody Valve
Implantation
Severe coronary
compression
36 year old – Melody Valve
Implantation
27 year old presents with
Advanced Stage Heart Failiure
• Dextrocardia
• L-transposition of the great arteries
• Pulmonary atresia with ventricular septal defect
– initial palliative shunt creations early in life (bilateral
modified Blalock-Taussig shunts Waterston shunt)
• Complete repair (age~7):
– LVOT reconstruction with a 19 mm aortic homograft
– VSD closure
– Complicated by complete heart block (dual chamber
pacemaker implantation)
27 year old presents with
Advanced Stage Heart Failiure
Hemodynamic History
• Declining Max VO2’s beginning—2002 (32 in
2000 to 22 in 2002)
• Clinical heart failure symptoms in—~2007
(LV [subpulmonary] systolic function mildly
depressed)
RA
LV
PA
PCW
RV
CI
3/09
7
75/6
17/9 (13)
6
95/9
--
8/10
21
--
--
--
--
2.0
10/11
21
77/21
25/15 (18)
13
78/17
1.89
12/12
18
81/19
30/18 (22)
16
78/16
1.74
Ventricular Status
LV
RV
RA
LA
Catheterization
Catheterization
Qp = 2.24 L/min -- 1.48 L/min/m^2
Qs = 2.64 L/min -- 1.75 L/min/m^2
Qp/Qs = 0.85:1
Rp (cannot be calculated due to
obstruction)
Rs = 23.11 Wood units -- 34.86 Wood
units-m^2
Conclusions
• Adults with all forms of congenital heart
disease require longterm follow-up
• Patients with complex congenital heart
disease require specialized care at a center
with established expertise in congenital
heart disease