Congenital Heart

Disease
dr Muhamad Taufik Ismail Sp.JP
Congenital heart disease

Incidence - 8/1000 live births

Most congenital defects are well tolerated
during fetal life.
Etiology
Unknown in most cases
Genetic factors
Chromosomal abnormality.
Environmental factors
 Type CHD
Lesions % of all Lesions
- Ventricular septal defect 25-30
- A trial septal defect (Secundum) 6-8
- Patent ductus arteriosus 6-8
- Coarctation of aorta 5-7
- Tetralogy of Fallot 5-7
- Pulomnary Valve Sterosis 5-7
- Aortic Valve Stenosis 4-7
- d-Transposition of great arteries 3-5
- Hypoplastic left ventricle 1-3
Classification:
acyanotic and cyanotic
Cyanotic classification
 Ventricular Defect

Small VSD
Asymptomatic
A loud, harsh, or blowing
holosystolic murmur.
Large VSD
dyspnea, feeding
difficulties, poor growth,
profuse perspiration,
recurrent pulmonary 80%
infections, and cardiac
failure in early infancy.
Atrial Septal Defects

In large defects, a
considerable shunt of
oxygenated blood flows
from the left to the
right atrium.
Mostly asymptomatic
The 2nd heart sound is
characteristically
widely split and fixed.

Secundum
Atrial Septal Defect

Enlargement of the right

ventricle
Enlargement of atrium
Large pulmonary artery
increased pulmonary
vascularity is.
Patent ductus arteriosus

Small defect no
symptoms.
Large defect:
Wide pulse
pressure
Enlarged heart
Thrill in L second
IS
Continuous
murmur
Tetralogy of fallot

Ventricular septal
defect
Pulmonic stenosis
Overriding aorta
Right ventricular
hypertrophy
Valvular Heart
Disease
dr Muhamad Taufik Ismail Sp.JP
 Type VHD
Mitral stenosis & Mitral
regurgitation
Aortic stenosis & Aortic
regurgitation
Pulmonary stenosis & Pulmonary
regurgitation
Tricuspid stenosis & Tricuspid
regurgitation
 Mitral stenosis

Etiology:
Rheumatic Fever (77-99%)
Congenital stenosis of the valve,
Prominent calcification,
Infective endocarditis
MITRAL STENOSIS PATHOPHYSIOLOGY

Mitral stenosis

Obstruction blood flow

To ventricle

High ventricle-atrial
pressure gradient

Elevation Hydrostatic pressure Decrease ventricle

In pulmonary vasculature Filling

Symptom of HF Low CO

Lilly L.S, 1998, Pathophysiology of Heart Disease

MITRAL STENOSIS
Clinical Presentation

Sign and symptom

Pulmonary congestion symptom
Low CO symptom, atrial fibrillation
Hemoptysis, hoarseness
Physical Finding
Loud S1, P2 load in PH
Opening snap, heard best in apex
Low pitch decrescendo, rumbling murmur,
heard best in apex
S1 loud
ECG-MS
X-ray

Convexity or
straightening LHB
Small aorta
Chepalization
Management

Medicamentosa
Slowing HR: beta blocker, digoxin
Congestion: diuretics

Intervention
Ballon mitral comissurotomy
Mitral valve repair/ replace
Mitral regurgitation

ETIOLOGY
Rheumatic deformity
Ischemic heart disease with papillary muscle
dysfunction
Infective Endocarditis
Idiopathic ruptured chordae
Mixomatous degeneration
Hypertrophic Cardiomyopathy
Marked left ventricular enlargement from any
caused
Mitral Regurg pathophysiology
Clinical manifestations

Symptoms
Fatigue and weakness
Dyspnea and orthopnea
Hemoptysis
Right sided HF
MVP Syndrome (if present)
Physical Exam
Holosystolic Apical Blowing Murmur
Laterally displaced apical impulse
Split S2 (but is obscured by the murmur)
S3 Gallop (increased volume during diastole)
Radiation depends on the etiology
MR - Management

Medikamentosa
Diuretics
Beta blocker
Vasodilator

Intervention
MVR/r
Mitral clip
Mitral valve operation
Aortic stenosis

Etiology: congenital, rheumatic, degeneratif

Aortic Stenosis pathophysiology
Clinical manifestation

Symptoms
Angina, syncope, HF
Physical findings
Ejection systolic murmur
Ejection click
Pulsus parvus et tardus
Paradoxical S2
S4,S3
Mechanism
Management AS

Medicamentosa
CHF: diuretics, vasodilator
Angina: beta blocker
Syncope: avoid dehydration

Intervention
AVR/r
Aortic regurgitation

Etiologies:
Abnormalities of the Leaflets: Rheumatic, Bicuspid,
Degenerative, Endocarditis
Dilation of the Aortic Annulus:
Aortic Aneurysm / Dissection
Inflammatory (Syphyllis, Giant Cell Arteritis.
Coll Vasc Dis-Ankylosis Spondylitis, Reiters)
Inheritable (Marfans, Osteogensis Imperfecta)
Sign and Symptoms: pulmonary congestion
symptom (chronic), sudden low CO
Physical:
Bounding pulse
Low diastolic pressure
Diastolic murmur, blowing, decrescendo, heard best in
left upper sternal border, Apical Rumble Austin Flint
Murmur
Aortic Regurg pathophysiology
Management

Medicamentosa
Vasodilator
Diuretics
Intervention
AVR/r
Pulmonary stenosis

8% of all CHD
Mostly asymptomatic
Ejection systolic murmur
Systolic click
Tx: Ballon valvuloplasty, surgery
Pulmonary Regurgitation

Mostly due to secondary cause

Heart Sound?
Tricuspid stenosis

Due to Rheumatic
Sound?
Tricuspid regurgitation

Pathological TR is more often functional rather

than due primary valve lesion
Functional lesion: annular dilatation,
secondary to: RV pressure & volume overload
Sound?
Questions?
Cor Pulmonale
dr Muhamad Taufik Ismail Sp.JP
Cor Pulmonale (CP) defined
Alteration of the right ventricular structure or
function that is due to pulmonary hypertension
(PHTn) caused by diseases affecting the lung
or its vasculature.
Excludes
Left sided heart disease with 2nd changes
Congenital heart disease
Etiologies

Pulmonary Artery vasoconstriction

Alveolar hypoxia
Blood acidosis
Anatomic redn of pulmonary vascular bed
Emphysema
Pulmonary emboli
Increased blood viscosity
Erythrocytosis (Includes polycythemia)
Sickle-cell disease
Increased pulmonary blood flow
Pathogenesis CP in COPD

COPD may develop late to Pulmonary hypertension and

is due mainly to hypoxic vasoconstriction of small
pulmonary arteries, eventually resulting in structural
changes that include intimal hyperplasia and later
smooth muscle hypertrophy/hyperplasia.
There is an inflammatory response in vessels similar to
that seen in the airways and evidence of endothelial cell
dysfunction.
The loss of the pulmonary capillary bed in emphysema
may also contribute to increased pressure in the
pulmonary circulation.
Progressive pulmonary hypertension may lead to right
ventricular hypertrophy and eventually to right-side
cardiac failure.
Patients with COPD

Most frequent cause of cor pulmonale

Right ventricular hypertrophy (RVH) in
40% of patients with FEV1 < 1.0 L
70% of patients with FEV1 < 0.6 L
Independent predictors of RVH
Hypoxemia
Hypercapnea
Erythrocytosis (not Polycythemia)
Prognosis of Cor Pulmonale

When due to COPD, PHTn plus peripheral

edema
5 year survival 30%, mean 3 years from dx
Pulmonary vascular resistance >550 dynes-sec/cm
rarely survive more than 3 years
May just reflect the degree of underlying COPD
Symptoms of CP

Directly attributable to PHTn

Dyspnea on exertion, fatigue, lethargy
Chest pain, syncope with exertion
Typical exertional angina
Occurs in patients with primary or secondary PHTn even in the
absence of epicardial CAD
Subendocardial RV ischemia induced by hypoxemia and
increased transmural wall tension
Dynamic compression of left main coronary by enlarged PA
Less common
Cough, hemoptysis, hoarseness
With severe right ventricular (RV) failure
Passive hepatic congestion
Anorexia, right upper quadrant discomfort
Physical Findings

Cardiac findings
RVH
Prominent A wave in the jugular venous pulse. with R sided 4th
heart sound
RV failure leads to systemic venous HTn
Elevated jugular venous pressure with a prominent V wave
RV S3
High pitched tricuspid regurgitant (TR) murmur
Extra cardiac changes
Hepatomegaly, pulsatile liver
peripheral edema-often related to hypercarbia and passive Na+
and water retention
Other Areas of Fluid Retention

Pleural effusion, often bilateral

Right heart failure until proved otherwise
Also kidney and liver
Engorged inferior vena cava
JVP
Hepatic congestion
Ascites
Anasarca
 Hepatojugular Reflux

Assessed by applying firm sustained pressure

over the upper abdomen with pt. breathing
quietly.
Response
Transient elevation by approximately 1 cm in normal
response
In RHF sustained elevation
Low specificity and sensitivity
 Radiograph in Cor Pulmonale

Enlargement of
Central PAs
In 95% of Pts
with PHTn from
COPD the
diameter of the
descending
branch of the
right PA is > 20
mm in width
Normal Chest Radiograph

Normal chest film

ECG in Cor Pulmonale
Management

O2
Treat underlying cause (lung problem)
Symptomatic therapy: diuretics
Supportive therapy; phlebotomy
Lung transplantation
Treatment

Oxygen
Relieves pulmonary vasoconstriction
Decreases PVR
Increases RV Stroke volume and cardiac output
Renal vasoconstriction may be relieved with increase in urinary
sodium excretion
Improves arterial oxygen tension with enhanced delivery to
Heart
Brain
Other vital organs (kidneys)
Long-term O2 administration has been shown to partially reduce
the progression of PH in COPD.
Treatment-Diuretics

Increasing RV filling volume using diuretics

Improve function of both RV and LV
As RV dilatation is reduced LV filling improves
May improve cardiovascular performance
Monitor for excessive volume depletion
BUN (blood urea nitrogen) Prerenal
Creatinine Renal
Estimated glomerular filtration rate (eGFR)
Watch for metabolic alkalosis
May suppress ventilation
Treatment, Continued

Digoxin is NOT indicated in pure CP

These PA Vasodilators are of NO benefit
Hydralazine
Nitrates
Nifedipine
Verapamil
Treatment with conventional vasodilators such as
CCBs is not recommended because they may impair
gas exchange due to the inhibition of hypoxic
pulmonary vasoconstriction and because of their
lack of efficacy after long-term use.
Theophylline/Terbutaline

Has effects other than direct bronchial

dilatation and diuresis
Improves myocardial contractility
Provides some degree of pulmonary
vasodilatation
Enhances diaphragmatic endurance
Narrow range of efficacy
Phlebotomy

When hematocrit > 55

Goal is hematocrit < 50
Secondary Erythrocytosis vs Polycythemia
Treat underlying condition
Sildenafil

Sildenafil: there is evidence that sildenafil

does not improve the results of rehabilitation
in patients with COPD and moderately
increased pulmonary artery pressure
Blanco I, et al. Sildenafil to improve respiratory
rehabilitation outcomes in COPD: a controlled trial.
Eur Respir J 2013 Oct;42(4):982-92.
Nitric oxide

In patients with COPD, in whom hypoxemia is

caused primarily by ventilation-perfusion
mismatching rather than by increased
intrapulmonary shunt (as in noncardiogenic
pulmonary edema), inhaled nitric oxide can
worsen gas exchange because of altered
hypoxic regulation of ventilation-perfusion
balance.
Therefore, based on the available evidence,
nitric oxide is contraindicated in stable COPD
 Infective
Encocarditis
dr Muhamad Taufik Ismail Sp.JP
Definition

IE is an endovascular, microbial infection of

intracardiac structures (valves, atrial or
ventricle endocardium).
Pathogenesis

a) Mechanical lesions (turbulent flow, electrodes or catheters,

inflammation, as in rheumatic carditis, or degenerative
changes in elderly) mechanical disruption of endothelium
exposure of underlying extracellular matrix proteins,
production of tissue factor, and deposition of fibrin and
platelets as a normal healing process (inflammation,
microulcers, and microthrombi) such nonbacterial
thrombotic endocarditis (NBTE) facilitates bacterial adherence
and infection.
b) Endothelial inflammation (without valve lesions) triggers
endothelial cells express integrins that bind circulating
fibronectin to the endothelial surface while S. aureus and
some other IE pathogens carry fibronectin-binding proteins on
their surface. Once adherent, S. aureus trigger their active
internalization into valve endothelial cells, where they can
either persist and escape host defences and antibiotics, or
multiply and spread to distant organs.
Diagnosis criteria
Treatment
Udaaaaahhhh..