Patient History
This is a case of G.L., a 21 year old female, complaining of easy fatigability and occasional chest pain who was referred for cardiovascular evaluation prior to employment. She was diagnosed to have “heart disease” in childhood. Past medical history revealed frequent upper respiratory tract infections.
Physical Examination
• General: Hypoesthenic, narrow A-P diameter
• Vital Signs: BP: 100/80 mmHg
PR: 75 bpm
RR: 20 cpm
BMI: 15 kg/m2
Physical Examination
• Thorax and Lungs:
symmetrical chest expansion, resonant on all lung all fields, (-) crackles
Physical Examination
• Cardiovascular Examination:
JVP and CAP – normal
at base: S1 is normal followed by a grade 3/6 crescendo
decrescendo murmur
S2 wide with fixed splitting
at apex: multiple clicks
12- Lead ECG
• Normal sinus rhythm
• Right ventricular hypertrophy
• Incomplete right bundle branch block
• Diffuse ST-T changes
2D- Echocardiography
• ASD, ostium secundum type• Markedly dilated right bentricle with adequate
wall motion and contractility with evidence of RV pressure and volume overload
• Dilated right atrium without thrombus• Dilated main pulmonary artery• TR, severe• PR• Moderate pulmonary hypertension• Reverse E/A across mitral valve
Salient Features
• 21 y/o, female
• Easy fatigability and occasional chest pain
• Childhood “heart disease”
• Frequent URTI
• Hypoesthenic with BMI of 15 kg/m2
PE CXR ECG 2D Echo
ASD, ostium secundum type
Left lower parasternal lift
Cardiomegaly with multi-chamber enlargement
Right ventricular hypertrophy
Dilated right ventricle with RV pressure and volume overload
Severe TR
Systolic grade 3/6 crescendo-decrescendo murmur at base
Dilated main pulmonary artery
Pulmonary regurgitation
PE CXR ECG 2D Echo
Fixed and widely split S2
Pulmonary congestion
Moderate pulmonary hypertension
Multiple clicks at apex
Reverse E/A across mitral valve
Cardiomegaly with multi-chamber enlargement
Right atrium and ventricle dilated
Diagnosis
• Cardiac Diagnosis:
Etiologic: Congenital Heart Disease
Anatomic:ASD, TR, PR
Dilated RA & RV,
Dilated Main Pulmonary Artery
Physiologic: not in failure
Functional: Class II-B• Pulmonary Hypertension
(+) upper respiratory tract infectionMitral stenosis
-dyspnea and cough on exertion
-orthopnea and PND
-hemoptysis
Mitral regurgitation
-dyspnea and cough on exertion
-orthopnea and PND
-ankle edema
premature birth, perinatal distress, or perinatal hypoxia may be present
most children with small defects remain asymptoma-tic
In patients with Eisenmenger syndrome, symptoms in adult life consist of exertional dyspnea, syncope and hemoptysis
R-L shunt leads to cyanosis, clubbing, and erythorocy-tosis
Prolonged symptom-free period
Palpitations
Fatigue
Dyspnea on exertion
Orthopnea
Frequent respiratory infections
Symptoms of right ventricular failure
“heart disease” in childhood
frequent URTI
easy fatigability
occasional chest pain
Hx
RHDPDAVSDASDPatient
Mitral stenosisdiastolic thrill at the apexS1 and P2 are accentuatedS2 is split or fixedOS of the mitral valve on expiration(+) carvallo's sign
Mitral regurgitation(+) systolic thrill at the apexholosystolic murmur -(+) S4
precordial activity is increased
apical impulse is laterally displaced
S1 normal, S2 typically obscured by murmur
Continuous machinery-like murmur
Bounding peripheral pulses
loud, harsh, or blowing holosystolic murmur is heard best over the lower LSB in the 3rd or 4th ICS
displaced cardiac apex with a similar holosystolic murmur
apical diastolic rumble and third heart sound (S3)
Prominent RV impulse
S1 normal or split, with accentuation of TV closure sound
Wide & fixed splitting of the S2
Systolic ejection murmur (heard in pulmonic area)
Diastolic rumble across the tricuspid valve
Neck vein distention
AscitesEdema
Hyposthenic, narrow AP chest diameter
Normal JVP and CAP
Left lower sternal lift
Normal S1 ff. by gr. 3/6 crescendo-decrescendo murmur
S2 wide with fixed splitting
Multiple clicks at apex
P.E.
RHDPDAVSDASDPatient
LVHLVH with a larger PDA
May be normal
With larger defects there are various degrees of right axis deviation associated with right ventricularEnlargement
Ostium secundum defects – incomplete right bundle block & right axis deviation
Ostium primum defects – left anterior hemiblock & left axis deviation
Normal sinus rhythm
RVH
Incomplete RBBB
Diffuse ST-T changes
ECG
RHDPDAVSDASDPatient
Mitral stenosis-(+) kerley B lines-concentric hypertrophy of left atrium-cephalization-prominent main pulmonary artery and branches-constriction of arteries in the middle and peripheral lung zones
Mitral regurgitation-eccentric hypertrophy of the left atrium-LA and LV enlargement-equalization, cephalization-mitral annulus calcification
LAE
LVE
PAE
PVE
RightVentricularenlargement
Increased pulmonary vascular markings
Shunt vascularity (inc. pulm. vascular markings)
Right ventricular enlargement
Enlargement of the pulmonary artery segment in P-A view
Cardiomegaly with multi-chamber enlargement and pulmonary congestion
CXR
RHDPDAVSDASDPatient
Mitral stenosis-mitral orifice <4cm-concentric hypertrophy of left atrium-annular calcifications
Mitral regurgitation-eccentric hypertrophy of the left atrium-LA enlargement-hyperdynamic LV-annular calcifications/ LV dyskinesis-ruptured chordae tendineae
LAE
Continuous flow from the aorta into the main pulmonary artery
color flow can show the shunting of blood from the leftventricle to the right VSD's can result in a shunt from below the tricuspid valve to below the pulmonary valve.
Enlargement of RV
Negative-contrast image at the site of defect (saline injection)
Doppler – abnormal pressure of left-to-right blood flow across the septum
ASD, ostium secundum type
Markedly dilated right ventricle with adequate wall motion and contractility with evidence of RV pressure and volume overload
Dilated RA w/o thrombus
Dilated MPASevere TR
PR
Mod. Pul. HPN
Reverse E/A across mitral valve
Echo
RHDPDAVSDASDPatient
Radiologic Findings
• L to R Shunt
ASD, Ostium secundum
Cardiomegaly
Accentuated pulmonary vascular markings
Pulmonary Arterial Hypertension
• Causes– Primary/ Idiopathic
• Genetic
– Secondary• Cardiac• Pulmonary
1. hypoxic vasoconstriction
2. decreased area of the pulmonary vascular bed
3. volume/pressure overload
Secondary Pulmonary Arterial Hypertension
• Hypoxic Vasoconstriction
– COPD and obstructive sleep apnea– Due to down regulation of endothelial nitric
oxide synthetase
• Decreased Area of Pulmonary Bed
– Occurs when loss of vessels exceed 60% of the total pulmonary vasculature
– Occurs in patients with collagen vascular disease like CREST and scleroderma. And those with chronic emboli
• Volume/ Pressure Overload
– Seen in patients with left to right intracardiac shunts
– May passively occur in patients with left atrial hypertension and left ventricular dysfunction, mitral valve disease and hose with aortic stenosis
Pulmonary Arterial Hypertension
• Common Features
– Medial hypertrophy– Eccentric and concentric intimal fibrosis– Recanalized thrombi– Plexiform lesions
There is medial hypertrophy and marked intimal hyperplasia of this muscular pulmonary artery. These changes are the morphologic response to increased pulmonary vascular pressures, such as may be seen in patients with chronic obstructive lung disease, pulmonary thromboembolism, or left-sided heart disease (e.g., mitral stenosis). Occasionally, elevated pulmonary pressures are seen without identifiable cause (idiopathic or primary pulmonary hypertension).
A. PA chest film demonstrates mild cardiomegaly with normal pulmonary arterial markings. There is pulmonary venous congestion, fluid within the horizontal fissure and prominent Kerley B lines (indicative of lymphatic engorgement).
Lateral chest film show marked venous congestion with fluid visible in both the horizontal and oblique fissures.
Pulmonary Arterial Hypertension
• Chest radiograph– The classic finding on a chest radiograph from
a patient with PAH is enlargement of central pulmonary arteries, attenuation of peripheral vessels, and oligemic lung fields.
– Findings of right ventricular and right atrial dilatation are possible.
A posterior-anterior (PA) chest radiograph demonstrates enlargement of the main pulmonary artery and right pulmonary artery .The peripheral pulmonary arteries are reduced in caliber. The lung
volumes are normal without evidence of underlying pulmonary parenchymal disease. A lateral radiograph also demonstrates enlargement of both the right and left pulmonary arteries.
Cardiomegaly, with predominant right ventricular enlargement is present.
Pulmonary Venous Hypertension
• Occurs secondary to increased resistance to pulmonary venous drainage
• Associated with diastolic dysfunction of the left ventricle and valvular dysfunctions (aortic and mitral valve stenosis and regurgitation)
Pulmonary Venous Hypertension
• Features– Capillary congestion– Focal alveolar edema– Dilatation of interstitial lymphatics
The alveolar septa appear thickened, and the alveolar spaces contain numerous pigment-laden macrophages. A few alveolar spaces contain pink edema fluid. Note the thick-walled pulmonary vessel.
Management
• Medical• Watch out for dev’t of complications
» Arrhythmias, CHF, endocarditis
• Operative repair: definitive– Patch of pericardium or of prosthetic material– Percutaneous transcatheter device closure– Indications:
• symptomatic• significant L to R shunting
– Qp:Qs ≥ 1.5:1 – RA or RV enlargement – radiographic, cardiac catheterization
• think otherwise if:– PVR > 12 U/mL / significant pulmonary hypertension– Eisenmenger – R to L shunting with cyanosis
Management
• Results• Surgical repair w/ mortality rates near zero• Complications: atrial arrhythmias, left atrial
hypertension • Transcather closure
– Air embolism (1-3%), thromboembolism from device (1-2%), systemic, pulmonary venous obstruction (1%), perforation of atrium/aorta (1-2%), atrial arrhythmias (1-3%)
Schwartz’s Principles of
Surgery, 8th Ed.