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

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

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 Past Medical History FAA Form 8500 Cardiac: Corrected transposition Medications lisinopril, digoxin, and amoxicillin Applicant maintains physically active lifestyle

5 Physical Exam Vitals: P: 62 bpm BP: 109/59 mmhg Ht: 72 inches (182 cm) Wt: 156 Ib (70 kg) Hearing, Vision, Dip stick unremarkable

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 Any pertinent, applicable studies

8 Past cardiac history: Cardiologist Report 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, corrected naturally, diagnosed in 2005

10 Medications: Cont., Cardiologist Report 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 Allergies: NKDA

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 aortic coarctation

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) with no evidence of cardiac limitation

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 Volume mildly decreased, EF 41%

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 8. Both aortic valve and mitral valve are normal

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 Holter monitor showed no significant ectopy

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 with an electrocardiogram and echocardiogram

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 Mitral 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 inversus 2 Multifactorial etiology Genetic, molecular and environmental 2% recurrence rate in first-degree relatives 3

25 Corrected Congenital Transposition of Great Vessels Total adults with congenital cardiac disorders now outnumber children Rare disorders primarily treated by pediatricians even through adulthood

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 age 4 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 echocardiogram and cardiologist evaluation

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 in this section

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 exam normal?

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 2. Heart 2010;96: Moss & Adams' Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult; 8 th Edition; pgs 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

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