Doc, I Am Fine, But I Have A Cardiac Condition

(1)

Nevine Mahmoud, MD

John Ludtke, MD

Maj, USAFR, MC, FS

RAM Class 2014

Wright State University Boonshoft School of Medicine Division of Aerospace Medicine

Dayton, OH

Doc, I Am Fine, But I Have A Cardiac

Condition

(2)

Disclosure

No financial disclosures or conflicts

of interest

(3)

Case History

30 y/o Caucasian male presents to our office

requesting a Third Class FAA Airman

Certificate

The applicant has been in good health and

has no complaints

(4)

FAA Form 8500

·

Past Medical History

·

Cardiac: Corrected transposition

·

Medications

·

lisinopril, digoxin, and amoxicillin

(5)

Physical Exam

·

Vitals:

· _{P: 62 bpm} · BP: 109/59 mmHg · Ht: 72 inches (182 cm) · Wt: 156 Ib (70 kg)

(6)

Cont., Physical Exam

·

Significant findings:

· _{Cardiac exam}

· RRR

· Holosystolic murmur with loud S2 · _{No scarring found on the chest wall}

(7)

Information Requested

·

PCP and cardiology records

·

Cardiovascular work up:

·

ECHO

·

Imaging studies

·

_Holter

·

Stress test

(8)

Cardiologist Report

·

Past cardiac history:

·

Corrected transposition (I, d, d)

·

Moderate RV function

·

EF 34% in 2008

·

Mild to moderate tricuspid insufficiency

·

Occasional brief palpitations, but better

·

Maintained on lisinopril and digoxin

(9)

Cont., Cardiologist Report

·

Review Of Systems:

·

Negative for cardiac symptoms except for

occasional palpitations

·

Past Medical History:

·

Transposition of the great arteries,

(10)

Cont., Cardiologist Report

·

Medications:

·

Digoxin 125 mcg tab po daily

·

Lisinopril 20 mg tab po daily

·

Amoxicillin 500 mg capsule PRN

·

Family History:

·

Unremarkable

(11)

Cont., Cardiology Report

Social History:

No tobacco use

Married, 2 sons and a daughter

Works at a midwestern AFB

(12)

Cont., Cardiologist Report

(Physical Exam)

·

Pertinent for Respiratory and Cardiovascular:

· _{No JVD}

· Mild pectus excavatum · Quiet precordium

· Mildly increased RV impulse · _{NL S1, single S2}

· 1-2/6 low pitched SEM at base

· 1-2/6 blowing systolic murmur LM-LLSB · Extremity pulses 2+ and equal

(13)

Laboratory / X-ray

· NT Pro BNP 70 (normal <125 pg/ml) · CBC and CMP normal

(14)

ECHO

1.

Mildly dilated right sided morphologic left atrium

2.

Atrial septum intact

3.

Tricuspid valve leaflets appear redundant, with

mild to moderate regurgitation (systemic AV valve)

4.

Trivial mitral regurgitation

5.

No aortic/pulmonary valve stenosis/regurge or

(15)

ECHO

6. Moderately dilated right ventricle

7. Right ventricular function moderately diminished 8. Moderate right ventricular hypertrophy

9. Quantitatively normal left ventricular function, with globular appearing apex and apical akinesis

10. Proximal RPA appears dilated, LPA was not adequately visualized

(16)

Metabolic Stress Test

Normal cardiopulmonary response to maximal exercise (RER 1.23, max HR, HR plateau)

(17)

MRI

· 1. Atrial and abdominal situs invertus · 2. Levocardia

· 3. Congenitally corrected transposition of the great

arteries

· 4. Mild to moderate tricuspid insufficiency, RF 16% · 5. Moderately hypertrophied RV with mild dilation

(18)

MRI

· 6. LV: Thin, D-shaped, apex hypokinetic, EF 44%

· 7. Mildly turbulent flow across the pulmonary valve.

Moderately dilated MPA and RPA, mildly dilated LPA. Without PA branch stenosis

(19)

Holter Monitor

·

Sinus Rhythm with 1

st

Degree A-V block, sinus

arrhythmia

·

Periods of sinus tachycardia

·

Maximum heart rate: 149 BPM

·

Rare ventricular ectopy as singles, couplets,

bigeminal cycles

·

Rare supraventricular ectopy as singles

·

No symptoms, per patient diary

(20)

Discussion

· _{Per Cardiologist the patient is doing very well}

clinically with no symptoms

· _{Did quite well on his exercise test with normal}

functional capacity

· Although he has some ventricular dysfunction, pro

BNP level normal

(21)

Discussion

The Cardiologist does not see any contraindication in allowing him to obtain a Third Class Airman’s Certificate No changes in his medications at that time

Recommendation to be seen and re-evaluated in one year

(22)

Diagnosis

·

Congenitally Corrected Transposition of the

Great Vessels (ccTGA)

· Atria in normal position

· Ventricles attached to wrong atria

· _{Great vessels are attached to the wrong ventricle} · _{“Double” congenital condition… one or the}

incompatible with life requiring fetal/neonatal cardiac surgery, but together are compatible

(23)

Corrected Congenital

Transposition of Great Vessels

http://www.nationwidechildrens.org/congenitally-corrected-transposition-great-vessels

Left

(24)

Corrected Congenital

Transposition of Great Vessels

·

Congenital heart defects 1% of births/year

1 · _{ccTGA 0.05% of congenital malformations}2

· Most associated with situs solitus · 5% associated with situs inversus2

·

Multifactorial etiology

· Genetic, molecular and environmental

(25)

Corrected Congenital

Transposition of Great Vessels

·

Total adults with congenital cardiac

disorders now outnumber children

·

Rare disorders primarily treated by

(26)

Corrected Congenital

Transposition of Great Vessels

·

Numerous Associated Lesions

· _{Most frequent:} · Septal defects

· Outflow obstruction · Valvular defects

· _{Anomalies of aortic arch}

· Conduction abnormalities dependent on details · ccTGA typically subtle on ECG

(27)

Aeromedical Concerns

· Adults w/ low mortality and good functional status

up to 40yrs of age4 · Long-term effects · Ventricular dilation/dysfunction · _{Valvular stenosis/regurgitation} · _Dysrhythmias · _{Heart block} · Tachyarrhythmias

(28)

FAA Disposition

·

Special Issuance Third Class medical

certificate granted

· Expires in 6 months pending CV evaluation. · Certificate extended annually pending stress

(29)

Waiver Experience

·

USAF – Congenital Heart Disease

· _{“Other, more complicated congenital disorders}

will be very unusual because most will be

detected in infancy or childhood and, even if

corrected, will be unacceptable for entrance into military service.”

· _{AIMWITS 2013 – ASD, VSD, PDA, PFO and}

coarctation of aorta present; no waivers found with search of terms “transposition” or “great vessel”

(30)

Waiver Experience

·

FAA

· _{“Defer conditions related to} · Cardiac decompensation

· Congenital heart disease accompanied by cardiac

enlargement, ECG abnormality, or evidence of inadequate oxygenation

· Hypertrophy or dilatation of the heart as

evidenced by clinical examination and supported by diagnostic studies

· Any other cardiac disorder not otherwise covered

(31)

Waiver Experience

·

USN

· _{Congenital cardiac issues not mentioned in}

medical standards or waiver guidance, only long term effects

· Cardiac decompensation · ECG abnormality

· Hypertrophy or dilatation · _{Valvular dysfunction}

· _{Possibility of flight duties if ECG and physical}

(32)

Summary

·

Corrected Congenital Transposition of the

Great Vessels (ccTGV) extremely rare

· Multitude of associated abnormalities

· Long term function of AV valve and systemic

ventricle most concerning

· _{Requires long term follow-up for symptoms of}

cardiac failure or dysfunction

· _{Normal physical exam and subtle ECG changes}

possible in rare cases at typical age of accession into military

(33)

References and Gratitude

· 1. CDC – Congenital Heart Defects

http://www.cdc.gov/ncbddd/heartdefects/data.html

· _{2. Heart 2010;96:1154-1161}

· _{3. Moss & Adams' Heart Disease in Infants, Children,}

and Adolescents: Including the Fetus and Young Adult; 8th Edition; pgs 1147-1160

· 4. J Thorac Cardiovasc Surg 2013 Mar 12

· Many Thanks to Dr. Arthur Pickoff, Chair Dept of

Pediatrics, Chair Dept of Community Health, Wright State University Boonshoft School of Medicine