Congenital cyanotic heart disease

congenital cyanotic heart disease

DDx - five "Ts"



90% of all  congenital heart defects are ToF (75%) or TGA (15%)

Treatment

TGA

PGE1 - keep PDA open

Fluids - needs volume for continued operation of atrial septal defect

Do not use oxygen -  want to keep pulmonary vascular resistance high

(+/- BAS)

Surgery: Switch

ToF

PGE1

+/- Oxygen

Fluids

Chromosomes - deGeorge syndrome

*Irradiate & wash blood*  -  kill white cells to avoid graph vs. host reaction

Surgery - Shunt or Repair

Total Anomolous Pulmonary Vascular Connection (TAPVC)

When should you NOT start with PGE1?  NEVER

Concerns re: PGE1

• Apnea
• Fever
• PVR
• O2 effect

PGE1 Dosing & PVR

PGE1 in obstructed TAPVC

PGE1 in TGA…& lasix. NEEDS FLUIDS

PDA

PDA Notes

#1 Determining factor – intervene or not – is…

Clinical trend line

“Severe” PAH ≠≠ elevated PVR  (REMEMBER P = Q x R)   inc.  P due to increased Q

Indomethacin - opposite of PGE1 = closes PDA (but side effects - close renal flow)

Indometacin (INN) or indomethacin (USAN and former BAN) is a non-steroidal anti-inflammatory drug(NSAID) commonly used as a prescription medication to reduce fever, pain, stiffness, and swelling. It works by inhibiting the production of prostaglandins, molecules known to cause these symptoms

CHF = congestive heart failure: clinical 

manifestations of inadequate cardiac output.

Differential Diagnosis

Primary

Myocardial
Rhythm
Blood 

Secondary

Shunt*
Obstruction
Both
A-V valve regurgitation

Acquired Heart Disease

Acquired Heart Disease

Kawasaki's Disease

 First described in Japan by Dr. Kawasaki in 1967.
 Toxic shock for younger patients?
• Immune response to a gram positive infection
 Self limited disease
 Most serious complication is the development of coronary artery aneurysms.
• Untreated 20 % incidence
• Treated with IVIGg, incidence goes down to 1-2 %
• Must treat within first 10 days of fever to be effective.


Kawashocki

Older age (>8 years)
Typically girls
Higher band counts and CRP, lower albumin
64 % are refractory to IVIG
Higher incidence of coronary artery aneurysms

Coronary Artery Aneurysms

• Typically seen in the first to weeks after the initial presentation.
• Of the patients that develop aneurysms
• ½ will regress back to normal
• More likely in patient < 1 year of age, small and fusiform in shape and distal.
• Of those that do not regress to normal
• ½ will enlarge and go on to giant aneurysms
• Giant aneurysms are at risk development of coronary thrombosis and stenosis leading to MI

Rheumatic Fever

Infection of the upper respiratory system by group A, beta hemolytic streptococci.

Toxin mediated immune response?

Diagnosis is made by using Jones criteria.

Rheumatic Heart Disease

Very common world wide

Less common in this country due to more aggressive management of strep pharyngitis

Still seen in this country however.

Children 5-15 years of age.

Shift to non-rheumtogenic strain leading to less classic presentations.

Jones Criteria

• Erythema Marginatum 3-5 %- Macular, non-pruritic with a serpiginous border. 
• Typically on thighs and trunk. Difficult to see on darker skin. Begins 1-2 weeks after initial infection. Usually gone when evaluated for other symptoms.
• Polyarthritis 80 %- Large Joints and migratory. Begins 2-5 weeks after infection. Painful and swollen. If just painful, not arthritis. Self-limited.
• Less responsive to ASA 
• Carditis 40-50 %(may be higher)- Typically valve changes. Mitral and aortic are the most common. Clinical symptoms early are typically only if myocardial or pericardial involvement. Valve involvement is the long term concern. Carey Coombs murmur.
• • Aschoff bodies in myocardium
•  Subcutaneous nodules 1%- Located on the extensor surfaces of joints- elbows, knees, hands and ankles. Freely movable 0.5 cm-2.0 cm.
• Sydenham’s Chorea (St. Vitus’s Dance)-Inflammation of the basal ganglia. 10-15%. Non-purposeful and rapid movements. Usually face and upper extremities. 6 months to a couple of years.

Treatment

• All patients get 10 days of Penicillin 
• If no or mild carditis
• • high dose ASA for 2-3 weeks, cut the dose in half and treat another 2-3 weeks.
• Moderate to severe carditis
• • Course of steroids with the addition of ASA when taper the steroids
• No strenuous activity until out of acute inflammatory stage.
• • Then based on severity of carditis

Heart Pain

Pericarditis

• Typically not a problem with systolic function.
• • Low stroke volume due to diminished preload 
• Entrapment of the heart limiting filling during diastole.
• • JVD and hepatomegaly
• Respiratory influence on filling more pronounced
• Decreased heart sounds or a rub on examination.

Don't treat with diuretics (need preload)

Myocarditis

• Viral etiology is most common
• • Children most often have entero (Coxsackie B) or adenoviris.
•  Almost any virus has been seen.
• • Patchy lymphocytic infiltrate with myoctye necrosis
• • Damage from pathogen and resultant immune response
• Other etiologies
• • Bacterial- Lyme’s disease, meningococcal, typhoid
• • Toxin mediated- C diptheria, S pyogenes, S aureus
• • Parasites- Typanosoma cruzi (chagas), toxoplasmosis, trichinella
•  Diagnostic Findings
• • Abnormal Echo in 98 %
• • Cardiomegaly on CXR in 60 %
• • Elevated troponin and ALT in 70-85 %
• • ECG changes noted in 93-100 % of cases.
•  ST flattening and T-wave inversion, low QRS voltages, axis deviation, LVH
•  Arrythmias
•  Major reason for death in these patients
•  Be attentive to low magnesium in these patients
• • MRI with gadolinium can detect patchy myocardial involvement.
• Treatment
• • Immune therapies have had limited data to show any clinical role.
  • IVIG may improve long term survival in chronic but no acute situation.
  • • Biopsy can at times be helpful in guiding management.
  • • Then again, maybe not due to patchy involvement
  • • Remember MRI

Note about pulse pressure; Histopathology review

Note about pulse pressure  (systolic minus diastolic BP)

Compliance = ΔV/ΔP

for arteries; compliance = ΔV=stroke volume; ΔP=pulse pressure

comliance = stroke volume/pulse pressure      so

pulse pressure = stroke volume/compliance

increased pulse pressure; e.g., 140/70 indicates either increased stroke volume OR decreased arterial compliance

Dr. Krinsky - histopathology review

lungs filter various types of emboli

amniotic fluid emboli produced during labor

inflammatory cells in asthma = eos

OBSTRUCTIVE LUNG DISEASE
• Emphysema
• Chronic Bronchitis
• Asthma
• Bronchiectasis

RESTRICTIVE LUNG DISEASE

• Pulmonary fibrosis

• Sarcoidosis

• Hypersensitivity pneumonitis

SMOKING RELATED DISEASE

• EMPHYSEMA

• DESQUAMATIVE INTERSTITIAL PNEUMONIA

• RESPIRATORY BRONCHIOLITIS

PULMONARY INFECTIONS

• ACUTE PNEUMONIA

• ASPIRATION PNEUMONIA

• VIRAL PNEUMONIA

• TUBERCULOSIS

• FUNGAL PNEUMONIA

foreign bodies in perivascular space come from injections; e.g., crushed oxycodone

THE HEART (rarely biopsied)

• Vessels
• Dissection
• Atherosclerosis
• Ischemic heart disease

• Acute myocardial infarction
• Chronic ischemic heart disease
• Coronary atherosclerosis
• Valvular disease
• Endocarditis = neutrophils  (myocarditits = lymphocytes)
• rheumatic heart disease

• Cardiomyopathy
• Hypertrophic cardiomyopathy
• Myocardium
• cardiomyopathy
• mycocarditis