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

Arteriosclerosis

Arteriosclerosis


Hardening and thickening of artery walls
• 3 types
– Atherosclerosis
– Medial Calcification
– Arteriolarsclerosis


Atherosclerosis
• Aorta - common
• Coronary Arteries - common
• Cerebral arteries - basilar and middle cerebral most affected
• Carotid arteries
• Abdominal arteries
– Splenic artery
• Peripheral arteries
• Pulmonary arteries - least common

Pathogenesis of Atherosclerosis

• Intimal injury - injury - markers for LDL.

– Aging

– Hemodynamic factors - turbulence; e.g., HTN

– High cholesterol

• Inflammation

– Monocyte adhesion and migration - turn into macrophages - eats debri = becomes foamy mac

– Oxidation of LDL

– Inflammatory response

monocytes become foamy macrophages after combining with LDL in plasma

Risk Factors

• Not changeable

– Age, Sex, Genetics

• Major reversible

– Smoking** Diabetes, HBP*, Hyperlipidemia

• Minor reversible

– Obesity, Life Style, Personality

*Hypertension
• Increases the severity of atherosclerosis at any age
• Mechanism is mechanical stress on arteries
– Intimal injury
– Medial degeneration

**smoking doubles or triples risk of sudden death


Clinical Effects of Atherosclerosis
• Arterial narrowing

• Bulging plaques
• Calcification
• Hemorrhage 

• Thrombosis

• Acute change in plaque
• – Erosion or ulceration
• – Plaque hemorrhage
• – Plaque rupture
• Vulnerable plaque
• – Moderate luminal narrowing
• – Lipid rich center
• – Thin fibrous cap

• Embolism

• Initiated by 
  rupture or erosion 
  of plaque
• Most from aorta
• – To Kidneys, Pancreas and 
  Spleen
• – To Lower limbs
• Dry gangrene

• Aneurysm - cystic medial degeneration - proteoglycan deposition - weakens walls

1. Atherosclerotic Aneurysm

• • Abdominal aorta belo w renal arteries
• • Iliac arteries
• • Usually over age 60
• • Men > women
• • Clinically significant when > 5 cm. in diameter

  2. Dissecting Aneurysm

• Hypertensive
•  Genetic
• – Marfan Syndrome
• Both types have cystic medial degeneration - 
• Dissection usually starts in arch of aorta

Coronary Artery Artherosclerosis

Myocardial Ischemia

• • Amount of coronary flow
• – Size/narrowing of coronary artery
• – Hypotension
• – Collaterals
• – Arrhythmias
• • Myocardial oxygen requirements
• – Anemia
• – Exercise/Excitement/Fever/Hyperthyroidism

Infarct Location

• Left anterior 

descending: Anterior 

wall, apex and 

septum

• Right: Posterior base 

and posterior septum

• Left Circumflex: 

lateral wall

Sequela of Infarct

• Death from arrhythmia or CHF

• Healing

• Aneurysm

• Rupture

• Postmyocardial syndrome

Sudden Cardiac Death
• Usually not Myocardial Infarct
• Mechanism is cardiac arrhythmia
• Thrombus not usually present
• Severe coronary narrowing
• Previous myocardial infarcts

Shunts

 Shunts

response to exercise = increased stroke volume = increased pulse pressure (Sys - Dia)BP

Qp/Qs  is the ratio of Fick principle calculations for pulmonary flow and systemic flow.
Qp = VO2/(PVO2 -  PAO2)

Qs = VO2/(CaO2 - CvO2)

when algebra is done and sats are used instead of content 
Qp/Qs = (AoSat - MVSat)/(PVsat - PAsat)

Coarctation of the Aorta

Pulses are difference between systolic and diastolic.   with collateral flow through parallel circuits; e.g., intercostal arteries, MAP can be normal but pulses are weak.

Coronary artery disease (CAD)

CAD

O2 supply vs. demand

supply = Flow x CaO2

• Q = P/R
• R - autoregulation; neural, local (adenosine)
• P - diastolic aortic pressure

demand = rate, inotropy, pressure, wall tension

• VO2 proportional to HR x Systolic BP (double product)

CAD

Fixed

• •Congenital anomalies
• •Vasculitides (collagen diseases)
• •Aortic dissection
• •Tumors
• •Scarring from trauma, radiation

Transient

• •Vasospasm
• •Embolus
• •Trombus in situ

Angina = Greek word ankhon (ἄγχω) meaning to strangle, throttle, or choke

• Classic (effort)
• Vasospastic or variant (Prinzmetal)
• Localized spasms associated with atheroma
• Unstable (medical emergency)
• Angina at rest or when it becomes longer or more frequent

Factors that Can Aggravate Myocardial Ischemia

Increased myocardial oxygen demand

• Tachycardia
• Hypertension 
• Thyrotoxicosis
• Heart failure
• Valvular heart disease
• Catecholamine analogues (eg, bronchodilators, tricyclic antidepressants)

Reduced myocardial oxygen supply 

• Tachycardia - reduces diastolic (flow) time
• Anemia
• Hypoxia
• Hypotension
• Smoking - produces HbCO

Treatment of CAD

1. Nitrates

Nitrates relax veins >>> arteries

• Increase venous capacitance
• Decrease preload
• Decrease pulmonary vascular pressure
• Heart size is decreased
• Decrease cardiac output
• Net:  decrease O2 demand, NOT increase O2 supply

•Dilate coronaries (normal individuals):

• Epicardial are sensitive (but not with concentric atheromas)
• Arterioles and precapillary spinchters are dilated least

Nitroglycerine is an arterial and venous dilator = reduces afterload and preload.

http://cvpharmacology.com/vasodilator/nitro.htm

Nitrates should not be used for right heart MI because reduced preload will worsen condition.

Adverse effects of nitrates

• Reflex tachycardia
• Increased myocardial O2 demand
• Decrease coronary perfusion (↓ diastole)
• Reflex increase in contractility
• Increased myocardial O2 demand
• formation of methemoglobin
• hypotension
• increase intracranial pressure
• tachyphylaxis (tolerance)

Other uses of nitrovasodilators

1.Hypertensive Emergencies: Na+ Nitroprusside

2.Congestive heart failure: isosorbide dinitrate + 

hydralazine

Congestive heart failure: isosorbide dinitrate + hydralazine (BiDil. Bi = two; Dil = dilators)

Na+ Nitroprusside

• Complex of cyanide, iron and nitrosomoiety

• Powerful, parentheral vasodilator. Rapid onset and dissapearance of action.

• Used for hypertensive emergencies and severe heart failure

• Dilates both veins and arteries

• Decreases peripheral resistance; ↔ or ↓cardiac output

• Rapidly metabolized in red blood cells; cyanide is metabolized by rhodanase, combined to less toxic thiocyanate in presence of a sulfur donor (thiosulfate). Thiocyanate is slowly eliminated by kidneys

• Side Effects:

1. Excessive decrease in blood pressure

2. Cyanide toxicity-to decrease risk co-administer thiocyanate or hydroxocobalamin

3. Thiocyanide toxicity in patients with renal failure (psychosis, seizures)

2. Beta blockers

-- ↓

Heart rate and 

contractility 

-- ↓

Cardiac Output

-- ↓

renin secretion

β1 selective blockers - name starts with letter A - M; e.g., metopralol = β1 blocker

Complications

1. bronchconstriction
2. decrease cardiac output
3. hypoglycemia
4. intermittent claudication
5. increase LDL decrease HDL
6. impotence
7. depression, sedation, nightmares
1. atenolol does not cross BBB - others do

The 70 year-old male patient being treated with dinitrate isosorbide returns to the 

family practice clinic because his angina pectoris symptoms have returned. He 

complains of palpitations. A decision is made to add metoprolol to his treatment 

regimen. The patient feels better a few days after the addition of this medication. 

What is the most likely mechanism responsible for the improvement in this 

patient’s condition:

A. Afterload reduction - no vasoconstricters

B. Coronary dilation - no beta receptors

C. Decreased heart rate

D. Increased renin levels  - decrease

E. Increased diastolic volume - not best choice

3. Ca channel blockers

Amlodipine (Norvasc (Pfizer) and generics) (as besylate, mesylate or maleate) is a long-acting calcium channel blocker dihydropyridine (DHP) class

• Slow, smooth onset of action
• •Long half life (once a day administration)
• •Antiatherogenic action (hyperlipidemia increases Ca2+
• influx to smooth muscle?). Decreased progression of 
• carotid atherosclerosis (but not coronary).
• •Reduced risk of major cardiovascular events
• •Minimum negative ionotropic effects (useful in patients 
• with LV dysfunction)

summary - angina drugs

4. Coronary revascularization

Compensatory Responses to Vasodilators - prevented by "polypharmacy"

Hypertension

here are a couple of really good concept figures from Katzung:

World Record Metabolic Acidosis

A 44-year-old woman drank approximately 720 ml of ethylene glycol in the form of antifreeze. She had previously attempted suicide by injecting the same substance into her buttocks. When admitted to the hospital, the patient was unresponsive and incontinent and was receiving ventilation. Her temperature was 37.1 °C (98.7 °F), her pulse 110 per minute, and her blood pressure 130/70 mm Hg. Her pupils were fixed and dilated, and she had no corneal, gag, or deep-tendon reflexes. The serum sodium level was 140 mmol per liter, the potassium level 6.6 mmol per liter, the chloride level 110 mmol per liter, and the bicarbonate level 1 mmol per liter (anion gap, 29 mmol per liter). The serum creatinine concentration was 2.8 mg per deciliter (248 μmol per liter), and the lactate concentration 10.1 mmol per liter. The osmolar gap (the difference between the measured value and the predicted value) was 84 mOsm per kilogram. Urinalysis revealed calcium oxalate crystals. The serum concentration of ethylene glycol was 2600 mg per liter; no other toxins were detected. Serial arterial-blood gas values are shown

Two hours after admission, hemodialysis was begun and continued intermittently for 48 hours. To reduce the conversion of ethylene glycol to its acid metabolites, ethanol was added to the dialysate1 and then given by gavage in a dose of 600 mg per kilogram of body weight, followed by intragastric infusion at a rate of 200 mg per kilogram per hour for 37 hours. Renal failure did not develop, and the patient recovered completely.


Although the arterial-blood pH of normal subjects may fall to 6.80 after extreme exertion,2 in a clinical setting such a level is usually fatal. Arterial-blood pH values of 6.78, 6.57, and 6.49 have been reported in patients who survived poisoning with ammonium chloride3 and strychnine4 and isoniazid overdose,5 respectively.

NEJM 328:515-516

85-YO male in ED

good article on medical response to Boston Marathon bombing


Drs. Voyles, Busby, Spaulding

85-YO male in ED

Diagnosis of COPD should be supported by objective data (e.g., PFT)


unable to assess CVP by JVD in obese patients


Cold upper and lower extremities; slight mottling of lower extremities = vasoconstriction

2/6 early SEM at the base only - could be due to turbulence from tachycardia, anemia, sclerotic valve leaflets

gallop uncertain - hard to tell at high heart rates S3 and S4 may blend with shortened diastolic period.  also hard to hear due to low pitch.  S4 occurs with atrial kick and LV hypertrophy from chronic HTN

Shallow respirations and decreased breath sounds in both bases; coarse rhonchi in all lung fields; scattered expiratory wheezes; no rales  -  rhonci = gunk in airways; shallow sounds = low volume breaths;  expiratory wheezing could be airway sounds or "cardiac asthma" where edema in lymphatics constricts airways = wheezing.  no rales or crackles could be due to shallow breaths and no opening of collapsed airways.  may become audible if pt. sits up and takes a big breath.

not making urine - maybe he voided in ambulance or is not making urine.  BPH may contribute.  ??? not making urine vs. not voiding.  hypotension responsible?
Place Foley catheter  and maybe bedside bladder scan.

Echocardiograms hard to obtain with morbidly obese pts.  transesophageal echo is a way around this.

don't give nl saline and furosemide at the same time.  need more data; e.g., blood pressure (via arterial line with pressure transducer in obese pts. since cuffs aren't accurate)  saline may increase 
BP BUT worsen pulmonary edema. 

Flank pain presenting complaint -  don't drift far from this; e.g., AAA
no abdominal tenderness since abdominal pain is peritoneal and AA is in peritoneal space.
CT scan problematic with high Cr.  Do it anyway if suspicion is AAA.

PE vs. AAA   -  in one case heparinize  not in AAA (would kill him)

Calcification occurs with smoking, obesity, and HTN


PE does not present with back and flank discomfort but AAA that ruptures does.
Tests for AAA

1. KUB xray
2. aortagram
3. CT scan - 

AAA can create ATN if renal arteries are involved.  

Huge surgical risks with this pt.  (not a candidate for a haircut)

option = endovascular repair  

pt. died on operating table with refractory shock.