Clinical Meeting On A young Adult with Clubbing & Cyanosis CHAIRPERSON: Dr. Md. Saiful Bari Associate professor & Head Dept. of Cardiology, MMCH Dept. of Cardiology, MMCH Speaker: Dr. Mahmud Hossain MD Student (Thesis part), Cardiology, MMC

Particulars of the patient  Name:Mr. Habibur Rahman  Age:36 Years  Sex:Male  Religion:Islam  Marital Status:Married  Occupation:Business  Address:Katbaula, Muktagacha, Mymensingh  Date of Admission :23/10/2011  Date of examination :23/10/2011

Presenting Complaints  Bluish discoloration of lips, tongue and fingers for 3 years.  Shortness of breath for 1 year.  Recurrent palpitation for 5 months.

History of present illness According to the statement of the patient he was reasonably well three years back. Then he noticed bluish discoloration of lips, tongue and fingers which aggravated on exertion. He also noticed gradual swelling of tips of fingers and toes. He developed shortness of breath which initially occurred with moderate to heavy exertion. At present it happens even at rest. Breathlessness was associated with fatigue and cough with frothy sputum but not blood tinged. It was not associated with chest pain. This symptom has no diurnal and seasonal variation. He gave history of occasional breathlessness at night.

History of present illness contd… The patient also complaints of palpitation for 05 months. Initially it was abrupt in onset and terminate spontaneously. But from the last two months it increased in frequency and duration. Palpitation was not associated with chest pain, and syncope but associated with dizziness and increased frequency of micturition. For these complaints he consulted with local physician but no improvement. With above complaints he got admitted into MMCH for better management.

History of past illness Patient gave no history suggestive of rheumatic fever and infective endocarditis. Prenatal, natal and post natal history could not be taken due to death of parents. Patient gave no history suggestive of rheumatic fever and infective endocarditis. Prenatal, natal and post natal history could not be taken due to death of parents.

Treatment History Patient took some medicine but cannot mention the name of drugs. Patient took some medicine but cannot mention the name of drugs.

Family History Parents not alive and died from natural cause. He has two brothers and two sisters, one son and one daughter. They are all alive and apparently healthy. Parents not alive and died from natural cause. He has two brothers and two sisters, one son and one daughter. They are all alive and apparently healthy.

Socioeconomic History Patient came from a poor socioeconomic family

Personal History He is smoker. He takes sticks/day for last 15 years. He is non alcoholic

Immunization History He was immunized against tuberculosis.

Physical Examination General Examination: Appearance- Ill looking Appearance- Ill looking Decubitus-Sitting Decubitus-Sitting Body build-Average Body build-Average Co-operation-Co-operative Co-operation-Co-operative Nutrition-Average Nutrition-Average Anemia-Absent Anemia-Absent Jaundice-Absent Jaundice-Absent

Physical Examination General Examination:  Cyanosis - Present (central)  Clubbing - Present in fingers and toes  Oedema-Present  Leuconychia-Absent  Pigmentation-Normal  Skin eruption-Absent

General Examination contd… Deformities- Absent Deformities- Absent Lymph node- Not palpable Lymph node- Not palpable Thyroid gland- Not enlarged Thyroid gland- Not enlarged JVP- Raised JVP- Raised Pulse beats/min (Irregularly irregular) Pulse beats/min (Irregularly irregular) Blood pressure- 90/70 mm of Hg Blood pressure- 90/70 mm of Hg Respiration- 25/min Respiration- 25/min Temperature F Temperature F Dehydration- Absent Dehydration- Absent

Systemic Examination  Cardiovascular system- Arterial pulse: Rate beats/min with pulsus deficit 50 beats/minute Rhythm- Irregularly irregular Volume & Character - Normal Symmetry - All peripheral pulses are bilateral symmetrically palpable Condition of the vessel wall – Normal Radio-Femoral delay – absent

Systemic Examination contd…  Examination of precordium :  Inspection:  Size & shape-Normal  Venous engorgement- Absent  Apex beat - Visible  Deformity or scar mark -Absent

Systemic Examination contd…  Palpation:  Apical impulse in left 6 th intercostal space 10 cm from mid sternal line, forceful and ill sustained.  Left parasternal heave – Absent  P 2 – Not palpable  Thrill – Absent

Systemic Examination contd…  Auscultation: o Heart sound: 1 st heart sound is soft. Pulmonary component of 2 nd heart sound is also soft. o Murmur : Ejection systolic murmur in the left 2 nd and 3 rd intercostal spaces radiating towards left clavicle, grade 3/6. A pansystolic murmur in the mitral area with left axillary radiation, grade- 2/6.

Systemic Examination contd…  Respiratory system : RR 25/min, Breath sound vesicular, Bi-lateral basal crepitation present.  Alimentary system : size & shape of abdomen normal, umbilicus centrally placed & inverted and liver is just palpable and tender.  All other systems : Reveals no abnormalities.

Salient Feature Mr. Habibur Rahman, 36 years, businessman, Married,normotensive,nondiabetic, nonalcoholic, smoker, hailing from katbaula, Muktagacha, Mymensingh admitted into this hospital with the complaints of bluish discolouration of lips, tongue and fingers for three years which aggravated on exertion. He also noticed gradual swelling of tips of fingers and toes. Mr. Habibur Rahman, 36 years, businessman, Married,normotensive,nondiabetic, nonalcoholic, smoker, hailing from katbaula, Muktagacha, Mymensingh admitted into this hospital with the complaints of bluish discolouration of lips, tongue and fingers for three years which aggravated on exertion. He also noticed gradual swelling of tips of fingers and toes.

Salient feature contd… Subsequently he developed shortness of breath for last one year, which was initially NYHA class –II but now NYHA class-iv. Breathlessness was associated with fatigue and cough with frothy sputum but not blood tinged. It was not associated with chest pain. This symptom had no diurnal or seasonal variation. He gave history suggestive of PND Subsequently he developed shortness of breath for last one year, which was initially NYHA class –II but now NYHA class-iv. Breathlessness was associated with fatigue and cough with frothy sputum but not blood tinged. It was not associated with chest pain. This symptom had no diurnal or seasonal variation. He gave history suggestive of PND

Salient feature contd… Patient also complaints of palpitation for 5 months. Initially it was abrupt in onset and terminate spontaneously. But for the last few months it increased in frequency and duration. Palpitation is not associated with chest pain and syncope but associated with dizziness and increased frequency of micturition. Patient also complaints of palpitation for 5 months. Initially it was abrupt in onset and terminate spontaneously. But for the last few months it increased in frequency and duration. Palpitation is not associated with chest pain and syncope but associated with dizziness and increased frequency of micturition.

Salient feature contd… He gave no H/o weakness of any part of the body. On general examination patient was ill looking, decubitus sitting position, patient has clubbing both in fingers and toes. cyanosis present in lips and tongue.

Salient Feature JVP raised and oedema present, no anaemia, jaundice, leuconychia, koilonychia. Pulse 130 beats/ min, irregularly irregular with pulse deficit 50 beats/min. JVP raised and oedema present, no anaemia, jaundice, leuconychia, koilonychia. Pulse 130 beats/ min, irregularly irregular with pulse deficit 50 beats/min.

Salient Feature BP- 90/70 mm Hg, RR 25/min, temperature 98 0 F. Cardiovascular system examination reveals Apex beat is in left 6 th intercostal space 10 cm from mid sternal line which is forceful and ill sustained. Left parasternal heave absent, no palpable P2 and thrill. BP- 90/70 mm Hg, RR 25/min, temperature 98 0 F. Cardiovascular system examination reveals Apex beat is in left 6 th intercostal space 10 cm from mid sternal line which is forceful and ill sustained. Left parasternal heave absent, no palpable P2 and thrill.

Salient feature contd… On auscultation S1 is soft pulmonary component of S2 also soft. Ejection systolic murmur in the left 2 nd and 3 rd intercostal spaces radiating towards left clavicle, grade 3/6. A pansystolic murmur in the mitral area with left axillary radiation, grade- 2/6.

Salient feature contd… On examination of respiratory system there is bi-lateral basal rales. On examination of alimentary system liver is just palpable and tender, no ascites. Examination of all other systems reveals no abnormality. On examination of respiratory system there is bi-lateral basal rales. On examination of alimentary system liver is just palpable and tender, no ascites. Examination of all other systems reveals no abnormality.

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Provisional Diagnosis Tetralogy of Fallot with MR with Atrial Fibrillation with Heart failure Heart failure

Differential Diagnosis  PS with VSD with heart failure with MR with AF  Eisenmenger’s syndrome with AF with heart failure  Ebstein’s anomaly with heart failure with AF  DORV with heart failure with MR with AF

Investigations: ECG 1: Lead I, II, III=>AF with fast ventricular rate.

ECG 2: Lead V1, V2, V3

Investigations ECG: AF with Fast ventricular rate ECG: AF with Fast ventricular rate Chest X-ray (P/A view): - Cardiac enlargement - Right sided pleural effusion

Investigations (contd.)  ECho-2D. M mode & colour doppler o Situs solitus, Levocardia o Single ventricle with double inlet and double outlet o Severe valvular pulmonary stenosis (PPG-85 mmHg) o MR (Gr-ll) o Good ventricular systolic function

Investigations (contd.)  Investigations not yet done o Cardiac catheterization o Coronary angiography o MRI

Investigations (contd.) Blood : Total count - 9,000 /cu mm Differential count : Neutrophils- 73% Lymphocytes- 22% Monocytes- 04% Eosinophils- 01% Hemoglobin (Hb) gm/dl ESR- 05 mm 1 st hour

Investigations (contd.) Peripheral blood film- RBC- Normocytic normochromic WBC- Mature Platelets- Normal in number Comments : Suggestive of secondary erythrocytosis.

Investigations (contd.) RBS mg/dl S.Creatinine- 1.2 mg/dl S. Electrolytes- Na mmol/dl K mmol/dl K mmol/dl Cl mmol/dl Cl mmol/dl

Dx Single ventricle with double inlet and double outlet with severe valvular pulmonary stenosis with MR Grade –II with heart failure with AF (Reverted)

Management 1. Medical Management: a ) Treatment of heart failure. a ) Treatment of heart failure. b) Anti-arrhythmic drugs. b) Anti-arrhythmic drugs. c) Prophylaxis against infective endocarditis. c) Prophylaxis against infective endocarditis.

Management 1. Surgical Management: a) Bidirectional Glenn’s operation. b) Modified Fontan operation.