Categorieën: Alle - symptoms - management - diagnosis - surgery

door Patrick Teloken 16 jaren geleden

591

Congenital Heart Disease

Congenital heart disease encompasses a variety of heart defects present from birth, classified into two main groups: acyanotic and cyanotic. Acyanotic defects include PDA, VSD, AVSD, and ASD, which often involve left-to-right shunts, leading to symptoms such as tachycardia, bounding pulses, and specific murmurs.

Congenital Heart Disease

Congenital Heart Disease

Cyanotic

Others
Truncus Arteriosus
Hypoplastic Left Ventricle
Totally Anomalous Pulmonary Venous Return
Tricuspid Atresia
Transposition of the Great Arteries

Mx:

Surgery within 2 weeks

Prostaglandin

CXR: "egg on a string" (narrow mediastinum)

Progressive cyanosis unresponsive to oxygen ==> less dramatic if VSD present

R-L Shunt
Ebstein´s anomaly

massively enlarged RA, patent foramen ovale ==> R-L shunt

Lithium / benzodiazepine use in 1st trimester

defect of the tricuspid valve ("atrialization of RV")

TOF

Mx: surgery

CXR: "boot-shaped" heart; decreased pulmonary flow

ECG: RAD, RVH

hypoxic spells caused by exertion

Mx: O2, knee-chest, morphine, propranolol

peak 2-4 months

may have L-R shunt initially; progressive RVOTO causes R-L shunt

VSD, RVOTO, overriding aorta, RVH

Murmurs

Pathologic
Unchanged with position
>3/6 +
Dyatolic ,pansystolic or continuous
Symptomatic
Innocent
varies with position
no extra sounds/clicks
systolic ejection
asymptomatic
do not warrant investigation!!!

General aspects

Risk Factors
Medications ==> Phenytoin, retinoic acid, valproate
Sibling with CHD
Alcohol ==> ASD, VSD
TORCH (Rubella)
SLE ==> complete heart block
DM ==> TGA
Turner ==> Aortic Coarctation, bicuspid aortic valve
Marfan / Ehler-Danlos ==> Mitral prolapse, aortic abnormalities
Down's Syndrome ==> AVSD
Prematurity ==> PDA
0,8% of births

Acyanotic

Obstructive
Pulmonic Stenosis

Mx: surgical repair

ECG: RVH

asymptomatic to CHF

usually part of other lesions

Aortic Stenosis

Mx: surgical correction; exercise restriction

Dyspnea + Syncope + Chest pain; ejection click

CoAo

Mx:

surgical/balloon correction

prostaglandin (keep ductus arteriosus open) for stabilization;

Complication: HTN

ECG: RVH in early infance; LVH later in childhood

often asymptomatic; higher SBP and stronger pulses in upper limbs

50% with bicuspid aortic valve; 35% Turner syndrome

pallor, decreased UO, cool extremities, poor pulses
Shunt L-R
AVSD

Mx: surgery by 6 months to prevent PAH

Spectrum from VSD and ASD to complete AV canal with common AV valve

common in Down´s syndrome

PDA

Mx: surgical/catheter closure if persistent after 3 months

ECG: LAH, LVH, BVH

tachycardia, bounding pulses, machinery murmur, hyperactive precordium

asymptomatic; may have apneic/bradycardic spells; poor feeding

VSD

Mod-Large

Mx: CHF management; surgical closure by 1 year

CXR: CHF features

ECG: LAD, LVH, RVH

2ary PAH; CHF by 2 months

small

most common CHD; asymptomatic ==> spontaneous closure

ASD

Mx: surgery/catether closure between 2-5 yrs

CXR: increased pulmonary vasculature

ECG: RAD, RVH, RBBB

CHF, PAH in adult life

Often asymptomatic; spontaneous closure if <8mm