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2.  - Circulation >> delivery of oxygen and
metabolites to various tissues >> maintain:-
 1- cellular integrity
 2- specialized function
 3- and growth


3.  Controlled by :-
 1- intrinsic control, predominantly by:-
 - The Frank-Starling mechanism
 - Autoregulation of peripheral vascular tone
 2- Extrinsic control, mainly through:-
 - Neural regulation of heart rate
 - AV conduction
 - Peripheral vascular resistance { afterload }
 - Peripheral venous capacitance { preload }

4.  Funamental determinants:-
 - Preload
 - Afterload
 - Intrinsic myocardial contractility

5.  Aim:-
 maintain adequate cardiac output
 Availability and efficacy differ depending on:-
 = age
 = underlying hear disease

6.  - C.O. = stroke volume X HR
 [ c.o. particularly HR-dependent in early
infancy >> so in presence of significant
heart disease >> bradycardia poorly tolerated
]
 - In infancy >> AV node less rate-limiting
>> HR up to 200 bpm are well tolerated

7.  - mainly under adrenergic control
 - In infancy, myocardium particularly
sensitive to negative-inotropic factors:
 = hypoxia
 = acidosis
 = hypocalcemia
 = hypoglycemia

8.  - Distribution is under neural and autoregulatory
or local control
 - When C.O. falls:-
 >>> blood flow to vital organs with greatest
metabolic demand [ heart and brain ] is
preferentially maintained.
 >>> increased adrenergic neurohumoral tone
>> reduces flow to skin, kidneys, GI tract >>
increases heart rate { in severe tachycardia >
pallor, cold skin, pyrexia and oliguria }

9.  - In presence of fever, sepsis, or anemia >>
associated with normal c.o.
 - Early signs: anxiety, restlessness, tachycardia
 cool and pale extremities
 - Early decompensation characterized by:
 tachypnea, metabolic acidosis, oliguria
 - Finally:
 impaired consciousness, hypotension and
periodic breathing

10.  - Presents most frequently in neonates and
young infants
 - Not unusual for mild circulatory failure to
precede an acute episode especially in
anomalies with left-to-right shunting
 - Dehydration, hypovolemia, anemia and
sepsis >> primary cause or make existing
HF worse

11.  - severe [ critical ] aortic stenosis
 - coarctation of aorta
 - aortic interruption
 - HLHS-hypoplastic lt heart syndrome-AS
 - Obstructed TAPVC
 - Persistent pulmonary hypertension
 - cardiac surgery
 - large VSD
 - Complete AVSD
 - Myocarditis or heart muscle disease
 - ALCAPA
 - Arrhythmias

12.  - Varied, complex, and depend on primary
etiology
 - Include:
 = LV systolic and diastolic dysfunction
 = Elevated LA &/or pulmonary venous pressure
 = Raised pulmonary artery pressure & PVR
 = RV systolic and diastolic dysfunction
 - Right HF & hepatic congestion occurs in the
majority
 - Emergency management is similar in most
groups

13.  - Rapid endo-tracheal intubation
 - Artificial ventilation with increased inspired O2
fraction
 - Intravenous sedatives, opiates, and muscle
relaxants
 - IV infusion of Prostaglandin E [if uncertainty
about CHD in newborn] >> duct-dependent
systemic circulation [ from MPA to AO ]

14.  - For neonates with persistent pulmonary
hypertension:
 = inhaled Nitric oxide
 = high inspired O2 levels
 = maintenance of low-normal systemic arterial
CO2
 = Very few >> ECMO support

15.  Persistent congestive HF:-
 - Choice of inotropic agent remains
controversial , with Dopamine or Dobutamine
used most frequently
 - Caution: use of large IV fluid volumes in
initial resuscitation [aggravating HF]

16.  Signs of therapeutic success :-
 - return of spontaneous urine output
 - resolution of metabolic acidosis
N.B.
some patients required IV diuretics [bolus or
infusion ]

17.  - Combination of fluid restriction with
diuretics carries risk of dehydration, and
should be used with caution
 - After stabilization : urgent treatment of
underlying heart malformation

18.  - Definition: A situation where symptoms such as
FTT, breathlessness or effort intolerance are
improved or remain stable with drug treatment.
 - If caused by un-correctable underlying
condition >> can lead to acute circulatory
collapse at any time.
 - Many infants or children with short-lived CHF
are best managed by surgical treatment of
underlying condition.

19.  - Excessive pulmonary blood flow
 - Ventricular volume overload [ semilunar or AV
valve incompetence
 - Recurrent or incessant arrhythmia
 - Primary myocardial failure
 N.B. :
 Congenital HD >> leading cause in developed
world

 Rheumatic HD >> predominates in developing
countries

20.  1- Acyanotic conditions with left-to-right shunt:
 = Complete AVSD
 = Moderate-to-large VSD
 = Moderate-to-large PDA

 - Commonest causes during first 6 months of life
 - Onset of symptoms usually from 4-6 weeks of age
[coincides with gradual fall of PVR after birth]
 - Onset earlier in premature infants [much more rapid fall in
PVR]
 - High PBF >> LA & LV overload >> pulmonary venous
congestion >> pulmonary edema >> dilated pulmonary arteries

21.  2- Valve insufficiency :
 = Aortic regurgitation
 = Mitral regurgitation
 = AV valve regurg. in AVSD
 - AR frequently seen in rheumatic HD
 - Congenital AR usually associated with bicuspid aortic valve
 - In Marfan syndrome >> ascending aortopathy >> AR
 - MR : common in rheumatic HD, dilated CMP, congenital
 - Mechanism : dysplastic leaflets, prolapse, isolated cleft, arcade
lesion.

22.  3- Cyanotic conditions without pulmonary
stenosis(TOF) or atresia(tricuspidatresia):
 = Common arterial trunk
 = DORV with sub-aortic or sub-pulmonary VSD
 = TGA with large VSD
 = Hearts with uni-ventricular AV connections
 = TAPVC

 - Cyanosis usually mild
 - Pulmonary blood flow increases as PVR gradually
falls after birth >> symptoms begin at 4-6 weeks

24.  Hearts with uni-ventricular AV connections

25.  4- Acquired heart disease:
 = Acute rheumatic carditis
 = Rheumatic HD
[ predominantly AR &/or MR ]

26.  5- Myocardial dysfunction:
 - Viral myocarditis
 - Kawasaki disease
 - Dilated CMP
 - Restrictive CMP
 - ALCAPA
 - Endocardial fibroelastosis
 - Transient myocardial ischemia of the newborn
 - Metabolic abnormalities
 - Carnitine deficiency
 - Hypocalcemia
 - Muscular dystrophy
 - Friedreich’sataxia

27.  6- Miscellaneous causes:
 - Incessant SVT
 - Neonatal CHB (congenital heart block)
 - Severe anemia
 - Acute hypertension
 - Acute cor pulmonale
 - Cardiac arrest with successful resuscitation

28.  1- Diuretics:
 - Mainstay of treatment
 - Usually used in combination [ spironolactone combined with
furosemide or chlorthiazide ]
 - Chlorthiazide is weaker and longer acting than furosemide
 - Major complication: dehydration and hypovolemia , electrolyte
loss in urine
 - Spironolactone unique in :
 1- potassium-sparing effect
 2- influence on myocardial remodeling due to aldosterone
antagonism and direct myocardial effect

29.  2- ACE inhibitors:
 - Used in combination with diuretics
 - Most frequently: captopril, enalapril, lisinopril [ single daily dose, not
used in infancy]
 - Action:
 1-reduce SVR & BP >> decrease left-to-right shunting + decrease
severity of MI/AI
 2- reduce LV pressure overload >> benefit patients with heart muscle
disease
 - Contraindicated in left heart obstructions
 - Complications: dizziness, hyperkalemia, skin rash, irritable dry cough,
and risk of potassium retention [ attention when used with
spironolactone ]

30.  3- Beta-blockers:
 - Carvedilol or Metoprolol, particularly patients with
relative resting tachycardia
 - Action: reduce HR >> increase ventricular filling
time >> improve c.o. >> improve symptoms
 - Contraindicated in:
 1- HR-dependent cardiac output patients,
 2- patients in whom beta-blockade further depresses
ventricular function >> sudden deterioration

31.  4- Oxygen:
 - The temptation to give supplemental
oxygen should be avoided in:
 1- left to right shunt
 2- increased PBF
 - Increased inspired oxygen concentration
>> drop in PVR >> marked increase in PBF
>> more severe congestive HF

32.  5- other treatments:
 - little evidence of any benefit from use of
digoxin or sodium restriction
 - Fluid restriction in combination with diuretics
in patient whose fluid intake already reduced [
SOB ] >> potentially dangerous >> lead to
dehydration >> acute deterioration
 - Heart failure with dehydration and hypovolemia
is potentially lethal

33.  6- Nutrition:
 - FTT and malnutrition
 - Also malabsorption
 - increase calorie intake
 - with or without NG, NJ, or even temporary
parenteral feeding may help