Congenital heart conditions Flashcards
Fill in the blank: Ebstein anomaly is characterized by _______ of the right ventricle due to a malformed tricuspid valve.
atrialization
What is Ebstein anomaly characterized by?
Atrialization of the right ventricle due to a malformed tricuspid valve
Severely affected newborns present with tricuspid regurgitation and cyanosis
What are the symptoms of severely affected newborns with Ebstein anomaly?
Tricuspid regurgitation and cyanosis
Some infants may have mild symptoms
Is Ebstein anomaly likely in a patient with a normal second-trimester ultrasound?
No
This diagnosis is unlikely in patients with normal ultrasounds
How is Ebstein anomaly
How is Ebstein anomaly diagnosed?
TTE.
What is the definative treatement for Ebstein anomaly?
Surgical repair.
What does hypoplastic left ventricle cause circulation to be dependant on?
Hypoplastic left heart syndrome will require the right ventricle to deliver both pulmonary and systemic circulation.
It is an embryologic malformation that occurs early in the first trimester
When is hypoplastic left heart usually discovered?
usually recognized on second-trimester ultrasound
What condition is hypoplastic left ventricle associated with in mothers?
Pregestational diabetes
hypoplastic left ventricle will presents with
cyanosis and is often respiratory distress with decreased peripheral pulses.
hypoplastic left ventricle will require immediate treatment with
Alprostadil and transcatheter septoplasty to maintain shunting followed by surgical repair.
hypoplastic left ventricle will be best diagnosed with … ?
TTE
What is coarctation of the aorta typically associated with?
Turner syndrome
What are the symptoms of neonates with severe coarctation of the aorta?
Weak femoral pulses and decreased postductal oxygen saturation
Aortic arch indentation (3 sign) is seen on radiography
What condition is congenital pulmonary valve stenosis typically associated with?
Noonan syndrome
Right ventricular outflow tract obstruction may result in cyanosis if right-to-left shunting occurs
Infants born to mothers with diabetes and poor glycemic control are at increased risk for what condition?
Transient hypertrophic cardiomyopathy
This is due to excess glycogen deposition in the fetal myocardium
What may be early signs of congestive heart failure in infants with hypertrophic cardiomyopathy?
Tachypnea and respiratory distress.
These signs may indicate heart failure
True or False: Hypoplastic left ventricle is often recognized on second-trimester ultrasound.
True
What congenital anomaly causes a narrowing (that usually just distal to the left subclavian artery) of the largest artery?
coarctation of the aorta
What is coarctation of the aorta?
A narrowing of the descending aorta, typically just distal to the left subclavian artery origin.
How does coarctation of the aorta present in newborns?
Tachypnea and respiratory distress. This usually occurs after closure of the ductus arteriosus, which is days after birth.
What is a major complication observed in neonates with severe coarctation of the aorta?
Weak femoral pulses
This indicates compromised blood flow
What are the clinical features of coarctation of the aorta?
Upper-lower body blood pressure differential, brachial-femoral pulse delay, upper body hypertension (headaches, epistaxis).
What congenital anomaly of the aortic arch results in the compression of the trachea or esophagus.
vascular rings
What congenital abnormality occurs with a left-to-right shunt that usually closes shortly after birth?
This is a Patent Ductus Arteriosus (PDA) is a communicating vessel between the aorta and pulmonary artery that remains open in utero due to low oxygen levels and elevated prostaglandin E2 (PGE2) levels. Normally, it closes shortly after birth when oxygen tension levels increase. If it remains patent, a left-to-right shunt occurs.
What are the risk factors for Patent Ductus Arteriosus (PDA)?
Risk factors for PDA include prematurity and maternal rubella infection during pregnancy.
What is the mechanism of action of indomethacin/ibuprofen in PDA closure?
Indomethacin and ibuprofen inhibit cyclooxygenase (COX), reducing prostaglandin E2 levels, which promotes closure of the ductus arteriosus. Contraindications relate to treatment that will make closure of the PDA worse.
What is an Atrial Septal Defect (ASD) and its types?
ASD is a congenital defect of the atrial septum. It is classified into two main types: Secundum, due to arrested growth of the septum secundum or excessive septum primum absorption, and Primum, caused by the failure of the septum primum to fuse with the endocardial cushions, often associated with atrioventricular canal defects.
What is the difference between secundum and primum ASD?
Secundum ASD results from abnormal development of the septum secundum or excess absorption of the septum primum. Primum ASD, associated with endocardial cushion defects, is commonly linked to Down syndrome.
What type of murmur is characteristic of ASD?
ASD is associated with a systolic ejection murmur at the pulmonary area (due to increased pulmonary blood flow), a fixed split second heart sound (S2), and a diastolic flow rumble murmur across the tricuspid area (due to increased right atrial blood flow).
