General Considerations Flashcards
Percentage of ACHD with complex cardiovascular anatomy
20%
Transition from pediatric to adult CHD program should start at what age?
12 years
Complete transfer from pediatric to adult CHD program should end at what age?
21 years of age
Anomalous left coronary artery from the pulmonary artery (ALCAPA)
Bland - White - Garland Syndrome
Pulmonary Hypertension with cyanosis due to right to left shunting
Eisenmenger Syndrome
Septal defect resulting in direct left ventricle to right atrium shunt
Gerbode Effect
Double inlet left ventricle with D-looped ventricles and normally related great vessels
Holmes Heart
Coronary sinus septal defect in the presence of left superior vena cava
Raghib Defect
Partial anomalous pulmonary venous connections of the right lower pulmonary vein to the IVC - RA junction, often accompanied by pulmonary artery hypoplasia and aortopulmonary collateral formation
Scimitar Syndrome
Series of left- sided obstructive lesions
Shone Syndrome
Form of double outlet right ventricle with D - malposed, side - by - side great vessels, sub - pulmonary VSD, hypoplastic aortic arch
Taussig - Bing Malformation
Early palliative procedure for transposition of the great arteries, with the inferior vena cava directed to the left atrium via homograft
Baffes Procedure
“Classic”—direct end to end anastomosis of subclavian artery to pulmonary artery
Blalock - Taussig - (-Thomas) Shunt
“Modified”—tube graft from subclavian artery to pulmonary artery
Blalock - Taussig (-Thomas) Shunt
Atriopulmonary anastomosis for single ventricle heart disease
Fontan or Fontan - Kreutzer
Closed infundibular resection for relief of pulmonary stenosis
Brock Procedure
Includes the right ventricle into the pulmonary circulation, was the unique modification for tricuspid atresia
Fontan - Björk Modification
“Classic”—end to end anastomosis of superior vena cava to right pulmonary artery
Glenn Procedure
“bidirectional”—end to side anastomosis of superior vena cava to right pulmonary artery
Glenn Procedure
Bidirectional Glenn in context of interrupted inferior vena cava with azygos continuation to the superior vena cava
Kawashima Procedure
Anterior translocation of the pulmonary arteries, so that both branch pulmonary arteries run anterior to the aorta. Most commonly used as part of the arterial switch operation
Lecompte Maneuver
Atrial switch operations for transposition of the great arteries, with atrial baffling using native ATRIAL tissue to redirect systemic and pulmonary venous flow
Senning
Atrial switch operations for transposition of the great arteries, with atrial baffling using native PERICARDIAL tissue to redirect systemic and pulmonary venous flow
Mustard Procedure
In double outlet right ventricle, posterior translocation of the aortic root towards the left ventricle, with baffling of the left ventricle to the aorta in its new position
Nikaidoh Procedure
Neonatal palliative procedure for hypoplastic left heart syndrome including aortic arch reconstruction with anastomosis of the native aorta to the pulmonary artery, which becomes the “neo-aorta,” as well as atrial septectomy and a modified BT shunt
Norwood Procedure
Direct anastomosis of the left pulmonary artery to the descending aorta
Potts Shunt
Intra-cardiac routing of the left ventricle to the aorta, which arose from the right ventricle. Usually accompanied by a right ventricle to pulmonary artery conduit.
Rastelli Procedure
Intrapulmonary baffle of the left coronary artery performed for anomalous left coronary artery from the pulmonary artery
Takeuchi Repair
Direct anastomosis of the right pulmonary artery to the ascending aorta
Waterston Shunt
How many percent of patients with ACHD have abnormal pulmonary tests?
40%
One of the leading causes of noncardiac death in ACHD patients
Pneumonia
Specific pulmonary complication in patients living with Fontan Physiology
Plastic Bronchitis
Hemoptysis may occur in upto _______ of ACHD patients with Eisenmenger Syndrome
1/3
Pulmonary hypertension is found in up to ____% of ACHD patients and is strongly associated with increased morbidity and mortality.
10%
Highest prevalence of impaired renal function.
Cyanotic ACHD
Second leading cause of non cardiovascular death in ACHD patients
Cancer
Malignancy associated with Fontan Physiology
Hepatocellular Carcinoma
Championed the segmental approach for description of congenital anatomy
Drs. Stella and Richard Van Praagh
In the normal heart the RV is connected to the PA by a ________________.
Complete Muscular Conus (Infundibulum)
In the normal heart, the LV is connected to the aorta by _____________.
Aortic-mitral fibrous continuity (without a complete conus)
The heart starts to form in the __________ weeks of gestation and is nearly fully formed by _________ weeks gestation.
3rd, 8th weeks
Cardiac neural crest cells migrate into the developing heart in the __________ weeks and are essential for septation of the outflow, formation of the semilunar valves, and patterning of the aortic arches.
5th to 6th weeks
Epidemiology of ACHD
Genetic or environmental cause can be identified in up to 30% of CHD cases. Single-gene disorders are found in 3% to 5%
Gross chromosomal anomalies/aneuploidy in 8% to 10%, and
Pathogenic copy number variants in up to 25% of CHD cases.
Environmental causes are identifiable in 2% of CHD cases.
The remainder of CHD is presumed to be multifactorial.
The most common aneuploidy and is usually caused by trisomy 21.
It is also the most common chromosome abnormality associated with CHD.
Down Syndrome
Fifty percent of children born with Down syn-drome have CHD, most commonly defects in the ______________.
AV Canal
Bile duct paucity, butterfly vertebrae, renal defects
Alagille Syndrome (JAG 1, Notch2)
Coloboma, choanal atresia, genital hypoplasia, ear anomalies, hearing loss, developmental delay, growth retardation, intellectual disability
CHARGE (CHD7)
Cleft palate, bifid uvula, velopharyngeal insufficiency, microcephaly, hypocalcemia, immune deficit, psychiatric disorder, learning disability
22q11.2DS (TBX1)
Skeletal dysplasia, short limbs, polydactyly, short ribs, dysplastic nails, respiratory insufficiency
Ellis - van Creveld Syndrome (EVC, EVC2)
Absent, hypoplastic, or triphalangeal thumbs; phocomelia; defects of radius; limb defects more prominent on left
Holt - Oram Syndrome (TBX5)
Common Atrium
Ellis - van Creveld Syndrome (EVC, EVC2)
Growth deficiency, wide palpebral fissures, large protuberant ears, fetal finger pads, intellectual disability, clinodactyly
Kabuki Syndrome (KMT2D, KDM6A)
Short stature, hypertelorism, down-slanting palpebral fissures, ptosis, low posterior hairline, pectus deformity, bleeding disorder, chylothorax, cryptorchidism
Noonan Syndrome (PTPN 11)
Vertebral anomalies, anal atresia, tracheoesophageal fistula, renal anomalies, radial dysplasia, thumb hypoplasia, single umbilical artery
VACTERL Association (unknown gene)
Unusual facies, thick lips, strabismus, stellate iris pattern, intellectual disability
Williams - Beuren (7q11/23 deletion or ELN)
In patients with TOF, ____________________ recorded on Holter monitoring predicts clinical VT.
asymptomatic nonsustained ventricular tachycardia (VT)
________________ is a risk factor for clinical VT and sudden death in patients with repaired TOF
Inducible sustained VT
Sinus node dysfunction is commonly encountered in patients with_____________ who have undergone a Mustard or Senning operation
D-loop TGA
Heart block is common in patients with___________ as the AV node is superiorly and anteriorly displaced.
L-loop TGA
The incidence of complete heart block in L-loop TGA is ______ % per year.
2%
the most common tachyarrhythmia in CHD, accounting for 62% of atrial arrhythmias.
Interatrial re-entrant tachycardia (IART)
What are the preferred antiarrhythmic in patients with complex CHD?
Sotalol and dofetilide
the most common atrial arrhythmia in adults with CHD older than age 50
Atrial Fibrillation
Most common cause of cardiac death in adults with CHD.
Accounts for upto 40% of ACHD Mortality
Heart Failure
Second most common cause of cardiac death in adults with CHD.
Accounts for approximately 20% of death in patients with CHD, occuring at a rate of ~ 0.1% per patient year
Sudden Death
Ascending aortic dilatation occurs in at least _______ % of patients with BAV
50%
Low-risk patients can begin a training program at approximately _____ % maximal predicted heart rate at least three times per week (total-ing 150 minutes/week) and increase intensity or duration over time.
70%
Criteria for Shunt Vascularity in Chest Radiograph
(1) uniformly distributed vascular markings with absence of the normal lower lobe vascular predominance;
(2) a right descending pulmonary artery diameter that exceeds 17 mm; and
(3) a pulmonary artery branch that is larger than its accompanying bronchus (best noted in the right parahilar area)
Prominent vascularity is apparent only if the pulmonary-to-systemic flow ratio is greater than _____ to 1.
As a rule, overt cardiac enlargement implies a shunt greater than ____ to 1
1.5:1, 2.5:1
The atrial situs follows the abdominal situs in ____________ of cases
70% to 80%
When both the aorta and inferior vena cava are on the same side of the spine, there is usually __________________.
abdominal and atrial right isomerism (two morphologic right atria)
Suspected when the intrahepatic inferior vena cava is interrupted, with the presence of azygos continuation in the paravertebral gutter, on either the left or right side.
Left atrial isomerism (two morphologic left atria)
