Download If chronic process – congestive heart failure

Non cyanotic
congenital heart diseases
Krzysztof Narebski
Torun
Problems to discuss
I.
•
•
•
•
Non cyanotic congenital heart diseases
(in theory) :
Coarctation of aorta
Aortic stenosis
Hypoplastic left heart syndrome
and
Truncus arteriosus communis
II. Heart failure
Changes in circulation from fetus to newborn. When congenital heart lesions
rely on blood flow through the duct (a duct-dependent circulation), there will
be a dramatic deterioration in the clinical condition when the duct closes.
Coarctation of the aorta
(CoA)
CoA - types
1. Duct dependant CoA - life threatening
malformation with symptoms of heart failure
from the neonatal period.
2. Non duct dependant CoA - with
hypertension in upper limbs, diminished
femoral pulses and systolic murmur
between shoulder blades.
Ancient terminology as preductal (infantile)
type and postductal (adult) type.
CoA - type 1
Juxtaductal location
– the most common
The systemic
circulation is
maintained by blood
flowing right to left
across the DA - a
duct-dependent
systemic circulation
and cyanosis !!!
CoA
type 2
The descending
aorta and its
branches are
perfused by
collateral channels
from the axillary
and internal thoracic
arteries through
the intercostal
arteries.
CXR
Murmur
ECG
CoA
CoA – signs and symptoms
1. Neonate with severe CoA – cardiovascular
collapse (pale, irritable, diaphoretic, dyspneic with
hepatomegaly and lack of femoral pulses).
Resemble sepsis !!! (icterus, oliguria, acidosis)
2. Older infants and children (mild CoA) –
hypertension in upper part (headache, chest
pain), hypotension in lower part (cold extremities,
leg claudication). After years heart failure and
aortic dissecting aneurysm can occur.
3. If female look for Turner syndrome !
CoA – cardiac examination
• Ejection (systolic) murmur over the coarctation –
usually left interscapular
area.
• Continuous murmur –
in case of extensive
collateral circulation
to the distal body
Normal
CoA – echo
CoA – chest x - ray
• If infants evolve to CHF - generalized cardiomegaly
with increased pulmonary vascular markings due to
pulmonary congestion.
• In older children and adults can occur:
- Notching of posterior one-third of the third to
eighth ribs due to erosion by large collateral
arteries (usually after the age of four). Not seen in
anterior ribs because the anterior intercostal
arteries are not located in costal grooves.
- A discrete indentation of aortic wall at site of the
coarctation plus prestenotic and poststenotic
dilatation can produce a "3" sign.
CoA – chest x - ray
Notching
”3” sign
CoA - treatment
1. Severe coarctation :
- prostaglandin iv to maintain ductal patency
- surgery as definitive treatment (resection with
end to end anastomosis)
2. Milder CoA or recurrent CoA after surgery :
- balloon angioplasty
- stenting (usually after balloon angioplasty)
Aortic stenosis
(AS)
AS - types
1. Valvular stenosis (usually due to bicuspid
aortic valve with prominent nodular masses
of calcium). The most common type.
1. Subvalvular (subaortic) stenosis.
1. Supravalvular stenosis.
Aortic
stenosis
AS. (b) Murmur. (c) Chest radiograph. (d) ECG.
AS – signs and symptoms
• In severe stenosis - duct dependant
systemic circulation – cardiovascular
collapse, cyanosis can occur
• Symptoms of congestive heart failure - pale
• Teenagers – sudden death (prolongation of
QT interval)
AS – cardiac abnormalities
• Ejection (systolic) murmur in aortic area.
• Cardiomegaly in
advanced disease.
• ECG LV hypertrophy
and LV strain
(repolarization abnormality)
in advanced disease.
AS – chest x - ray
White arrow
- poststenotic
dilatation
Red arrow
- left
ventricular
enlargement
AS
- CT
Calcification
of the aortic
valve
AS - echocardiography
• Valvular stenosis : dismorphic bicuspid or
unicuspid valve, annular hypoplasia
• Subvalvular (subaortic) stenosis :
1. fixed stenosis because of fibrous diaphragm
2. dynamic obstruction because of myocardial
hypertrophy
• Supravalvular stenosis: rare
AS - treatment
• If severe stenosis – prostaglandin iv
• Percutaneous balloon valvuloplasty or
surgery as definitive treatment
Hypoplastic left heart
syndrome
(HLHS)
The entire left side of the
heart is underdeveloped!!!
1. Hypoplastic left ventricle
2. Hypoplastic aortic arch
(CoA can occur)
3. Hypoplastic / stenotic
mitral and aortic valves
4. PDA and PFO supplies
blood to systemic
circulation
HLHS – signs and symptoms
Signs of heart failure up to cardiogenic shock
(cardiovascular collapse) when closure of DA :
• Hypothermia
• Weak pulses
in all extremities
• Tachycardia
• Respiratory distress
• Pallor up to cyanosis
• Hepatosplenomegaly
• Usually no cardiac murmur
HLHS - ecg
•
•
•
•
•
Sinus tachycardia
Right axis deviation
Right atrial enlargement
Right ventricular hypertrophy
Paucity of left ventricular forces in left
precordial leads
Chest x-ray (A) and
ECG (B) of newborn on the first day of life.
HLHS - echo
HLHS - treatment
Lethal if not surgically addressed!
Before surgery :
• Prostaglandin iv infusion
• Carefully with oxygen (lower PVR)
• Carefully with diuretics
(if pulmonary overcirculation)
• Ionotropes can be successful
Surgery as definitive treatment (up to heart tx)!
Truncus arteriosus
communis
(TAC)
TAC
1. Single arterial trunk
2. Normal ventricles
3. Single semilunar valve
4. Small or absent DA
(not required for fetus)
TAC
Different types
of pulmonary
arteries origin.
TAC – other possible features
• Structural abnormalities of the truncal
valve, (dysplastic and supernumerary
leaflets).
• Regurgitation (moderate or severe)
through the truncal valve.
TAC - pathophysiology
Combination of heart failure and cyanosis.
• Qp > Qs typically 3-fold higher (lower PVR).
• Pulmonary overcirculation and heart failure.
• Mixing of left and right
ventricular output at
common trunk resulting
in subnormal systemic
arterial oxygenation.
TAC – signs and symptoms
• Heart failure (poor feeding, diaphoresis,
tachypnea, tachycardia)
• Cyanosis
• Progressive metabolic acidosis and
myocardial dysfunction, which can result in
multiple organ failure
• Association with DiGeorge syndrome
TAC - murmurs
VSD large – equalization of
pressures = no VSD murmur.
Murmurs can be:
 Diastolic (regurgitation)
through common valve.
 Systolic if stenotic pulmonary
artery. Cyanosis rather than
palor.
TAC
Cardiomegaly with increased pulmonary vascularity
TAC - ecg
• Biventricular hypertrophy
• Left atrial enlargement (patients with
substantial pulmonary overcirculation)
TAC echo
TAC MRI
TAC - treatment
• Without surgery almost 100% mortality by
the age of 1 year.
• Digitalis and diuretics for congestive heart
failure.
• Surgery as definitive treatment.
Recapitulation
Common features for CoA, AS, HLHS and
Interrupted aortic arch according to degree
of left ventricular outlet obstruction (LVOTO)
are :
1. Cardiovascular collapse (severe) - duct
dependant systemic circulation, hence
cyanosis can occur !!!
2. Congestive heart failure (less severe)
with pallor !!!
In TAC there is heart failure (pallor),
sometimes also cyanosis, but no LVOTO !
Treatment for CoA, AS, HLHS and Interrupted
aortic arch with severe left ventricular outlet
obstruction (LVOTO) and cardiovascular collapse
(closure of DA) is :
Prostaglandin (PGE1) continuous iv infusion 0.1 –
0.01 mcg/kg/min
In TAC PGE1 is
not administered!
Cyanosis develops
either :
by desaturation of arterial blood (central
cyanosis)
or :
by increased extraction of oxygen by
peripheral tissues (peripheral cyanosis), as
occurs in severe heart failure or shock.
Heart failure
Heart failure - abbreviations
•
•
•
•
•
•
•
CHF – congestive heart failure
PVR – pulmonary vascular resistance
SVR – systemic vascular resistance
CVP – central venous pressure
RAAS - renin-angiotensin-aldosterone system
Qp – pulmonary blood flow
Qs – systemic blood flow
Heart failure vs. shock
Definition of heart failure :
Clinical syndrome characterized by
systemic perfusion inadequate to meet the
body's metabolic demands (oxygen and
nutrients) as a result of impaired cardiac
pump function.
Definition of shock : Inability of cardiovascular
system to provide tissues with oxygen
(oxygen deficit) plus hypotension.
Heart failure vs. shock
Types of shock :
1. Hypovolemic - dehydration, bleeding
2. Distributive - anaphylactic, septic or trauma
(there is not net hypovolemia but improper
distribution of fluid)
3. Cardiogenic ~ cardiovascular collapse
Heart failure progresses gradually in
opposition to cardiogenic shock which is a
sudden illness !!!
Heart failure - types
1. Mechanical dysfunction :
- Systolic overload (pressure) – systemic or
pulmonary hypertension, CoA, aortic or
pulmonary stenosis
- Diastolic overload (shunt or valve
insufficiency) : AVSD, VSD, ASD
2. Filling (of ventricles) dysfunction :
- mitral or tricuspid stenosis, tamponade
3. Direct cardiac cell injuries :
- hypoglycemia, asphyxia, toxins, infarction
Heart failure – right ventricle
Increased end diastolic pressure and volume in RV :
• Enlargement of RV
• Tricuspid valve insufficiency (murmur)
Increased pressure in right atrium and
Increased central venous pressure (CVP) hence :
• Hepatosplenomegaly
• Pleural, peritoneal or pericardial effusion (rare)
• Edemas (palpebral, limbs)
• Cyanosis (deceleration of blood flow, lower
venous saturation, polycythemia)
Heart failure – left ventricle
Increased end diastolic pressure and volume in LV :
• Enlargement of LV
• Mitral valve insufficiency (murmur)
Increased pressure in LA – arrhythmia, atrial flutter
Increased pulmonary venous pressure hence :
• Dyspnoea (shortness of breath)
• Crepitations on pulmonary auscultation
If chronic process – congestive heart failure !!!
If sudden process – pulmonary edema !!!
Heart failure – cardiac etiology
Neonates – obstructed systemic circulation :
- Hypoplastic left heart syndrome
- Critical aortic valve stenosis
- Critical CoA, interrupted aortic arch
Infants – enlarged pulmonary blood flow :
- AVSD, VSD, Large ASD and PDA
Older children and adolescents :
- Eisenmenger syndrome
- Rheumatic heart disease
- Cardiomyopathy
Heart failure – non cardiac etiology
- Neonatal sepsis, asphyxia, hypoglycemia,
hypocalcaemia
- Tachyarrhythmia
- Respiratory insufficiency (e.g. pneumonia,
air leak syndrome)
- Toxic injury (e.g. cocaine)
Heart failure
Symptoms (breathlessness on feeding or exertion) :
- Sweating
- Poor feeding (failure to thrive)
- Recurrent chest infections
Signs :
- Tachypnea / tachycardia
- Heart murmur, gallop rhythm
- Enlarged heart
- Hepatosplenomegaly
- Cool peripheries
Shortness of breath
Especially in congestive heart failure
• PVR much less than SVR
(pulmonary pressure < systemic pressure)
• VSD, ASD, AVSD associated with left to right shunt
and increased pulmonary blood flow, which lead to:
vasculature engorged and intestinal edema,
leading to excess fluid in the lung tissue causing a
barrier for gas exchange
As compensation tachypnea and respiratory effort.
As complication respiratory infections.
Fatigability and failure to thrive
Children with CHF (due to left to right shunt) cannot
feed and grow well b/o:
1. Overstimulation of catabolism (catecholamines,
RAAS) and suppresion of anabolism (insulin).
2. Shortage of substrates (oxygen and nutrients).
Heart failure - treatment
According to etiology !!!
- calcium or oxygen administration, pleural
drainage, antibiotics, surfactant, etc.
- surgery (CoA, AVSD, VSD, TAC, AS, PS)
and
Symptomatic treatment !!!
HF – symptomatic treatment
• Limit exertion, tube feeding
• Fluid restriction
• Oxygen (carefully in left to right shunt; can
increase shunt and diminish systemic load !)
• If severe - reduce energy expenditure
(mechanical ventilation, parenteral nutrition)
HF – symptomatic treatment
1. Diminish overload (diuretics) :
spironolactone, furosemide
2. Diminish neurohormonal reaction :
Catecholamines & RAAS can injure cardiac cells.
(ACE inhibitors, betablockers - carefully in infants)
3. Augment cardiac output (ionotropes):
digoxin; in severe CHF (catecholamines –
dobutamine, dopamine and noradrenaline; or
phosphodiesterase inhibitor - corotrope)
Thank you for your attention