Clinical Meeting Clinical Meeting

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Clinical Meeting Clinical Meeting

OnOn

A young Adult with Clubbing &

Cyanosis Cyanosis

CHAIRPERSON:

Dr. Md. Saiful Bari Dr. Md. Saiful Bari

Associate professor & Head Associate professor & Head Dept. of Cardiology, MMCH Dept. of Cardiology, MMCH

Speaker:

Dr. Mahmud Hossain Dr. Mahmud Hossain

MD Student (Thesis part), Cardiology, MMC MD Student (Thesis part), Cardiology, MMC

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Particulars of the patient Particulars of the patient

 NameName :: Mr. Habibur RahmanMr. Habibur Rahman

 AgeAge :: 36 Years36 Years

 SexSex :: MaleMale

 ReligionReligion :: IslamIslam

 Marital StatusMarital Status :: MarriedMarried

 OccupationOccupation :: BusinessBusiness

 AddressAddress :: Katbaula, Katbaula, Muktagacha,

Muktagacha, Mymensingh Mymensingh

 Date of Admission Date of Admission :: 23/10/201123/10/2011

 Date of examination Date of examination :: 23/10/201123/10/2011

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Presenting Complaints Presenting Complaints

 Bluish discoloration of lips, tongue Bluish discoloration of lips, tongue and fingers for 3 years.

and fingers for 3 years.



Shortness of breath for 1 year.Shortness of breath for 1 year.



Recurrent palpitation for 5 months.Recurrent palpitation for 5 months.

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History of present illness History of present illness

According to the statement of the patient he was According to the statement of the patient he was reasonably well three years back. Then he reasonably well three years back. Then he noticed bluish discoloration of lips, tongue and noticed bluish discoloration of lips, tongue and fingers which aggravated on exertion. He also fingers which aggravated on exertion. He also noticed gradual swelling of tips of fingers and noticed gradual swelling of tips of fingers and toes. He developed shortness of breath which toes. He developed shortness of breath which initially occurred with moderate to heavy initially occurred with moderate to heavy exertion. At present it happens even at rest.

exertion. At present it happens even at rest.

Breathlessness was associated with fatigue and Breathlessness was associated with fatigue and cough with frothy sputum but not blood tinged.

cough with frothy sputum but not blood tinged.

It was not associated with chest pain. This It was not associated with chest pain. This symptom has no diurnal and seasonal variation.

symptom has no diurnal and seasonal variation.

He gave history of occasional breathlessness at He gave history of occasional breathlessness at

night.

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History of present illness contd…

The patient also complaints of palpitation for 05 The patient also complaints of palpitation for 05 months. Initially it was abrupt in onset and months. Initially it was abrupt in onset and terminate spontaneously. But from the last two terminate spontaneously. But from the last two months it increased in frequency and duration.

months it increased in frequency and duration.

Palpitation was not associated with chest pain, Palpitation was not associated with chest pain, and syncope but associated with dizziness and and syncope but associated with dizziness and increased frequency of micturition. For these increased frequency of micturition. For these complaints he consulted with local physician complaints he consulted with local physician but no improvement. With above complaints he but no improvement. With above complaints he got admitted into MMCH for better management.

got admitted into MMCH for better management.

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History of past illness History of past illness

Patient gave no history suggestive of Patient gave no history suggestive of rheumatic fever and infective endocarditis.

rheumatic fever and infective endocarditis.

Prenatal, natal and post natal history could Prenatal, natal and post natal history could

not be taken due to death of parents.

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Treatment History Treatment History

Patient took some medicine but Patient took some medicine but

cannot mention the name of drugs.

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Family History Family History

Parents not alive and died from natural Parents not alive and died from natural cause. He has two brothers and two

cause. He has two brothers and two sisters, one son and one daughter.

sisters, one son and one daughter.

They are all alive and apparently They are all alive and apparently

healthy.

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Socioeconomic History Socioeconomic History

Patient came from a poor socioeconomic Patient came from a poor socioeconomic

family family

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Personal History Personal History

He is smoker. He takes 10-15 sticks/day for last He is smoker. He takes 10-15 sticks/day for last

15 years. He is non alcoholic 15 years. He is non alcoholic

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Immunization History Immunization History

He was immunized against tuberculosis.

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Physical Examination Physical Examination

General Examination:



AppearanceAppearance -- IllIll looking looking



Decubitus_Decubitus -- SittingSitting



Body build_{Body build} -- AverageAverage



Co-operationCo-operation -- Co-operativeCo-operative



Nutrition_Nutrition -- AverageAverage



Anemia_Anemia -- AbsentAbsent



Jaundice_Jaundice -- AbsentAbsent

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Physical Examination Physical Examination

General Examination:



_Cyanosis_Cyanosis

- -

Present (central)Present (central)



ClubbingClubbing - Present in fingers and - Present in fingers and toes

toes



OedemaOedema -- PresentPresent



LeuconychiaLeuconychia -- AbsentAbsent



PigmentationPigmentation -- NormalNormal



Skin eruptionSkin eruption -- AbsentAbsent

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General Examination contd…

DeformitiesDeformities - Absent- Absent

Lymph nodeLymph node - Not palpable- Not palpable

Thyroid glandThyroid gland - Not enlarged- Not enlarged

JVPJVP - Raised- Raised

_Pulse_Pulse

- -

130 beats/min 130 beats/min

(Irregularly irregular) (Irregularly irregular)

Blood pressureBlood pressure - 90/70 mm of Hg- 90/70 mm of Hg

RespirationRespiration - 25/min- 25/min

TemperatureTemperature - 98- 98⁰⁰FF

DehydrationDehydration - Absent- Absent

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Systemic Examination Systemic Examination

 Cardiovascular system-Cardiovascular system-

Arterial pulse:

RateRate - 130 beats/min with pulsus - 130 beats/min with pulsus deficit 50 beats/minute

deficit 50 beats/minute Rhythm

Rhythm - Irregularly irregular- Irregularly irregular Volume & Character

Volume & Character - Normal- Normal Symmetry

Symmetry - All peripheral pulses are - All peripheral pulses are

bilateral symmetrically palpable bilateral symmetrically palpable Condition of the vessel wall – Normal

Condition of the vessel wall – Normal Radio-Femoral delay – absent

Radio-Femoral delay – absent

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Systemic Examination contd…

 Examination of precordium : Examination of precordium :

 Inspection: Inspection:



Size & shapeSize & shape -- NormalNormal



Venous engorgementVenous engorgement - - AbsentAbsent



Apex beat Apex beat - - VisibleVisible



Deformity or scar mark -Deformity or scar mark - AbsentAbsent

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Systemic Examination contd…

 Palpation: Palpation:

Apical impulse in left 6Apical impulse in left 6^th^th

intercostal space 10 cm from mid intercostal space 10 cm from mid

sternal line, forceful and ill sternal line, forceful and ill

sustained . sustained .

Left parasternal heave – AbsentLeft parasternal heave – Absent

 PP₂₂ – Not palpable – Not palpable

Thrill – AbsentThrill – Absent

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Systemic Examination contd…

 Auscultation: Auscultation:

o Heart sound: 1Heart sound: 1^st^st heart sound is soft . heart sound is soft . Pulmonary component of 2

Pulmonary component of 2^nd^nd heart sound heart sound is also soft .

is also soft .

o Murmur : Ejection systolic murmur in the Murmur : Ejection systolic murmur in the left 2

left 2^nd^nd and 3 and 3^rd^rd intercostal spaces radiating intercostal spaces radiating towards left clavicle, grade 3/6. A

towards left clavicle, grade 3/6. A

pansystolic murmur in the mitral area with pansystolic murmur in the mitral area with

left axillary radiation, grade- 2/6.

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Systemic Examination contd…

Respiratory systemRespiratory system : RR 25/min, : RR 25/min, Breath sound vesicular, Bi-lateral Breath sound vesicular, Bi-lateral

basal crepitation present.

Alimentary systemAlimentary system : size & shape of : size & shape of abdomen normal, umbilicus centrally abdomen normal, umbilicus centrally

placed & inverted and liver is just placed & inverted and liver is just

palpable and tender.

All other systemsAll other systems : Reveals no : Reveals no abnormalities.

abnormalities.

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Salient Feature Salient Feature

Mr. Habibur Rahman, 36 years, businessman, Mr. Habibur Rahman, 36 years, businessman, Married,normotensive,nondiabetic,

Married,normotensive,nondiabetic,

nonalcoholic, smoker, hailing from katbaula, nonalcoholic, smoker, hailing from katbaula, Muktagacha, Mymensingh admitted into this Muktagacha, Mymensingh admitted into this hospital with the complaints of bluish hospital with the complaints of bluish discolouration of lips, tongue and fingers for discolouration of lips, tongue and fingers for three years which aggravated on exertion. He three years which aggravated on exertion. He also noticed gradual swelling of tips of also noticed gradual swelling of tips of

fingers and toes.

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Salient feature contd…

Subsequently he developed shortness of Subsequently he developed shortness of breath for last one year, which was initially breath for last one year, which was initially NYHA class –II but now NYHA class-iv.

NYHA class –II but now NYHA class-iv.

Breathlessness was associated with fatigue Breathlessness was associated with fatigue and cough with frothy sputum but not blood and cough with frothy sputum but not blood tinged. It was not associated with chest pain.

tinged. It was not associated with chest pain.

This symptom had no diurnal or seasonal This symptom had no diurnal or seasonal

variation. He gave history suggestive of PND variation. He gave history suggestive of PND

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Salient feature contd…

Patient also complaints of palpitation Patient also complaints of palpitation

for 5 months. Initially it was abrupt in for 5 months. Initially it was abrupt in onset and terminate spontaneously.

onset and terminate spontaneously.

But for the last few months it But for the last few months it increased in frequency and duration.

increased in frequency and duration.

Palpitation is not associated with Palpitation is not associated with chest pain and syncope but chest pain and syncope but associated with dizziness and associated with dizziness and

increased frequency of micturition.

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Salient feature contd…

He gave no H/o weakness of any He gave no H/o weakness of any

part of the body. On general part of the body. On general

examination patient was ill examination patient was ill

looking, decubitus sitting looking, decubitus sitting

position, patient has clubbing position, patient has clubbing

both in fingers and toes. cyanosis both in fingers and toes. cyanosis

present in lips and tongue.

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Salient Feature Salient Feature

JVP raised and oedema present, JVP raised and oedema present, no anaemia, jaundice,

no anaemia, jaundice,

leuconychia, koilonychia. Pulse leuconychia, koilonychia. Pulse

130 beats/ min, irregularly 130 beats/ min, irregularly

irregular with pulse deficit 50 irregular with pulse deficit 50

beats/min.

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Salient Feature Salient Feature

BP- 90/70 mm Hg, RR 25/min, BP- 90/70 mm Hg, RR 25/min, temperature 98

temperature 98⁰⁰F. Cardiovascular system F. Cardiovascular system examination reveals

examination reveals Apex beat is in left Apex beat is in left 66^th^th intercostal space 10 cm from mid intercostal space 10 cm from mid

sternal line which is forceful and ill sternal line which is forceful and ill

sustained. Left parasternal heave sustained. Left parasternal heave

absent, no palpable P2 and thrill.

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Salient feature contd…

On auscultation S1 is soft pulmonary On auscultation S1 is soft pulmonary component of S2 also soft. Ejection component of S2 also soft. Ejection

systolic murmur in the left 2

^nd^nd

and 3 and 3

^rd^rd

intercostal spaces radiating towards intercostal spaces radiating towards left clavicle, grade 3/6. A pansystolic left clavicle, grade 3/6. A pansystolic murmur in the mitral area with left murmur in the mitral area with left

axillary radiation, grade- 2/6.

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Salient feature contd…

On examination of respiratory system On examination of respiratory system there is bi-lateral basal rales. On

there is bi-lateral basal rales. On

examination of alimentary system liver examination of alimentary system liver

is just palpable and tender, no ascites.

Examination of all other systems Examination of all other systems

reveals no abnormality.

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. . ??? ??? ? ? ? ? ? ?

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Provisional Diagnosis Provisional Diagnosis

Tetralogy of Fallot with MR with Tetralogy of Fallot with MR with

Atrial Fibrillation with Atrial Fibrillation with

Heart failure Heart failure

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Differential Diagnosis Differential Diagnosis

PS with VSD with heart failure with MR with PS with VSD with heart failure with MR with AFAF

Eisenmenger’s syndrome with AF with heart Eisenmenger’s syndrome with AF with heart failure

failure

Ebstein’s anomaly with heart failure with AFEbstein’s anomaly with heart failure with AF

 DORV with heart failure with MR with AFDORV with heart failure with MR with AF

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Investigations:

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

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ECG 2: Lead V1, V2, V3

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Investigations Investigations

ECG: AF with Fast ventricular rate ECG: AF with Fast ventricular rate Chest X-ray (P/A view):

Chest X-ray (P/A view):

- Cardiac enlargement Cardiac enlargement

- Right sided pleural effusionRight sided pleural effusion

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Investigations (contd.) Investigations (contd.)



ECho-2D. M mode & colour dopplerECho-2D. M mode & colour doppler

o

Situs solitus, LevocardiaSitus solitus, Levocardia

o

Single ventricle with double inlet and Single ventricle with double inlet and double outlet

double outlet

o

Severe valvular pulmonary stenosis Severe valvular pulmonary stenosis (PPG-85 mmHg)

(PPG-85 mmHg)

o

MR (Gr-ll)MR (Gr-ll)

o

Good ventricular systolic functionGood ventricular systolic function

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Investigations (contd.) Investigations (contd.)

 Investigations not yet done Investigations not yet done

o Cardiac catheterization Cardiac catheterization

o Coronary angiography Coronary angiography

o MRI MRI

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Investigations (contd.) Investigations (contd.) Blood :

Blood :

Total count -

Total count - 9,000 /cu mm9,000 /cu mm Differential count :

Differential count : Neutrophils

Neutrophils - 73%- 73%

Lymphocytes

Lymphocytes - 22%- 22%

Monocytes

Monocytes - 04%- 04%

Eosinophils

Eosinophils - 01%- 01%

Hemoglobin (Hb)

Hemoglobin (Hb) - 16.7 gm/dl- 16.7 gm/dl ESRESR - 05 mm 1- 05 mm 1^st^st hour hour

(37)

Investigations (contd.) Investigations (contd.)

Peripheral blood film Peripheral blood film- -

RBC- Normocytic normochromic RBC- Normocytic normochromic

WBC- Mature WBC- Mature

Platelets- Normal in number Platelets- Normal in number

Comments :

Comments : Suggestive of secondary Suggestive of secondary erythrocytosis.

erythrocytosis.

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Investigations (contd.) Investigations (contd.)

RBS RBS - 6.5 mg/dl - 6.5 mg/dl

S.Creatinine

S.Creatinine - 1.2 mg/dl- 1.2 mg/dl S. Electrolytes

S. Electrolytes - Na- Na⁺⁺ 125 mmol/dl 125 mmol/dl KK⁺⁺ - 3.8 mmol/dl - 3.8 mmol/dl ClCl^-^- - 98 mmol/dl- 98 mmol/dl

(39)

Dx Dx

Single ventricle with double inlet Single ventricle with double inlet

and double outlet with severe and double outlet with severe

valvular pulmonary stenosis with valvular pulmonary stenosis with

MR Grade –II with heart failure with MR Grade –II with heart failure with

AF (Reverted)

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Management Management

1. 1. Medical Management Medical Management : :

a a

) Treatment of heart failure. ) Treatment of heart failure.

b) Anti-arrhythmic drugs.b) Anti-arrhythmic drugs.

c) Prophylaxis against infective c) Prophylaxis against infective endocarditis.

endocarditis.

(41)

Management Management

1. 1. Surgical Management Surgical Management : :

a)a) Bidirectional Glenn’s operation.Bidirectional Glenn’s operation.

b)b) Modified Fontan operation.Modified Fontan operation.