Pulmonary Arterial Hypertension

1. Median survival rate in  idiopathic PAH - 

• 2.8 YEARS

2. % of median survival in IPAH  in NIH registry - 

• 1 year - 68 %
• 3 year - 48%
• 5 year - 34 %

3 . Indian/ japan studies suggest median survival in IPAH -

• 2-3 years

4. worst prognosis in PAH  seen in cases of 

• PAH associated with scleroderma 
• 2  year survival  40%

5. congenital heart disease with  EMS  

• prognosis is better than IPAH
•  SURVIVAL RATE - 
• 1 YEAR -97 % 
• 2 YEAR - 89 % 
• 3 YEAR  - 77 % 

6. poor prognosis in PAH  indicated by - 

• C/F RV failure 
• rapid progression of symptoms 
• Class IV   functional status 
• 6 minute walk test < 300 m
• Peak VO2 Max < 10.4 ml / kg/ min
• ECHO - 
• pericardial effusion
• RV  enlargement
• RA enlargement
• RVdysfunction
• RA  pressure > 20 mmHg 
• cardiac index  < 2.0   l /min/sq. m
• BNP significantly elevated

7.

TETRALOGY OF FALLOT

1. INDICATIONS of Intervention In  Repaired TOF -  

• Residual VSD >  1.5
• residual significant  PS
• RV pressure > 1/3 rd  of systemic pressure 
• severe PR -  with RV  dilation 
• RV  diastolic volume index  > 170 ml/sq m
• QRS  > 180 ms
• Symptomatic AR
• Aortic root size > 55mm 

2  . INDICATION  of  pulmonary valve replacement  in Repaired  TOF  

•  Asymptomatic  + 2 or more criteria
• symptomatic + 1 or more criteria
• TOF repair for > or  = 3 years + > or = 1 criteria
• woman + severe PR + RV dilation  + RV dysfunction + + > or = 1 criteria

•  Quantitative criteria -

  • RVEDVI> 150 ml/sq m

  •  z score > 4

  • RVEDV/LVEDV >2

  •  RVEDVI> 80 ml / sq m

  • RVEF > 47 %

  • LVEF<55%

  • Large RVOT aneurysm

  • QRS > 140 ms

  • RVOT obstruction with RV Systolic pressure  > or = 2/3 rd of systemic pressure

  • severe branch PA stenosis 

  • Moderate TR

  •  Sustained tachy arrythmia

3 . cyanosis in TOF

• Cyanosis may be apparent in the perinatal  period, or somewhat later in infancy.

• cyanosis has been noted within the first six months of life in 75 to 90% of infants

• cyanosis and hypoxic spells  that develop after the perinatal period are usually

associated with

progressive muscular infundibular hypertrophy, as well as  growth failure of the right  ventricular outflow tract due to diminished pulmonary blood flow.

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4 .  Repaired TOF prognosis  -

• 1-2 % operative mortality
• good survival  for 20 years
• reoperation needed for 10- 15 % cases in 20 years
• survival > 90%  in 20 years

5 . Predictors of death in TOF  repair - 

• Old age of repair
• QRS > 180 ms
• PR SEVERE  - ASSOCIATED WITH  - Sudden Cardiac Death
• TR - SEVERE  - ASSOCIATED WITH  - SVT
• increased CT ratio > 0.6
• Multiple VPCS 

6.  TOF  consists of -

• (1) stenosis of the pulmonary artery; 
• (2) interventricular communication; 
• (3) deviation of the origin of the aorta to the right; and
• (4) right ventricle   hypertrophy, almost always concentric

7. TOF  -   right aortic arch is found in 

• 25% of cases

8.  Other abnormalities frequently seen in TOF -

• Coronary artery abnormalities include an origin of the left anterior descending coronary artery (LAD) from the proximal right coronary
• subclavian artery may arise from a retroesophageal location in up to 10% of cases
• persistence of the left superior vena cava - 10% of cases
• mitral valve hypoplasia

9. Confluence and arborisation  of pulmonary arteries 

• Central pulmonary arteries are confluent in approximately 75% of cases; however, they may supply only a portion of each lung because of the presence of MAPCAs and arborization abnormalities. 
• Arborization abnormalities are more common and more severe with nonconfluent pulmonary arteries, but occur in up to 50% of patients with confluent pulmonary arteries

10.Genetics of  TOF - 

• Microdeletion of chromosome 22q11 is the most common deletion,
• Microdeletions in the region of 22q11 occur in 
• 83% of individuals with velocardiofacial syndrome, 
• 94% of those with DiGeorge syndrome, 
• 20% of patients with TOF, 
• 40% with TOF/PA. 
• Fluorescent in situ hybridization techniques allow for rapid detection of this microdeletion.

11. Describe the oxygen saturation / shunt  according to severity of RVOT obstruction in TOF -

• Mild RVOTO- 
• They have signs and symptoms of pulmonary overcirculation due to a left-to-right shunt at the VSD. 
• Oxygen saturations are normal ( “pink tetralogy”), 
• congestive heart failure (CHF) occurs with the normal decrease in pulmonary artery pressures during the first few weeks of life.

• Moderate RVOTO  - 
•  near-normal pulmonary-to-systemic blood flow ratio (Q_p:Q_s) 
•  oxygen saturations in the low 90s
•  severe  RVOTO - 
• significant right-to-left shunting occurs at the VSD, 
• oxygen saturations are typically in the 70s

 12. Hypercyanotic episodes -

• occur because of a change in the pulmonary-to-systemic blood flow ratio (Q_p:Q_s). 

• A severe hypercyanotic episode is characterized by 

• irritability, 
• hyperpnea, 
• marked cyanosis, 
• pallor, and 
• lethargy or unconsciousness  

• known as a “tet spell.” 

• A decrease in systemic vascular resistance or an increase in pulmonary vascular resistance leads to increased right-to-left shunting and marked desaturation.
• The desaturation and resulting metabolic acidosis can increase the pulmonary resistance, thereby worsening the right-to-left shunting

 13 . Describe the physiology in TOF PA -

• Physiology in TOF/PA depends on the amount of PBF. 

• Because all PBF is derived from the systemic circulation,

• pulmonary systemic oxygen saturations will be equal. 

• The greater the PBF, the higher the systemic saturation. 

• Saturation levels greater than 85% indicate a Q_p:Q_s of 2:1 or greater, 

• saturations in the 70s indicate a Q_p:Q_s of 1 or less. 

• As in TOF, patients may have evidence of not enough, adequate, or too much PBF. 

• Decreasing arterial oxygen saturations in the neonate may indicate that the ductus arteriosus is closing. 

• High oxygen saturations indicate excessive PBF and will lead to CHF. 

• This high flow through nonrestrictive MAPCAs may lead to pulmonary vascular obstructive disease in the affected lung segments.

14 .  Natural history  of TOF - 

Dependent on the severity of the anatomic defect. 

• Mortality rates are 
• 30% at 6 months 
• 50% by 2 years. 
• < 10% can be expected to reach age 21 years. 

Patients with TOF/PA have a much worse prognosis,

• Mortality rate of 50% by 1 year 
• 85% by age 5 years.

PDA

1. Ductus arteriosus develops from which arch -

• 6th arch

2.  Ductus arteriosus  closes by =

• anatomical closure - 2-3 hours
• physiological closure - 2-3 weeks

3.Ductus dependent circulation  -

• Ductus dependent pulmonary circulation - 

•  tricuspid atresia
• pulmonary atresia with  intact ventricular septum

• Ductus dependent systemic  circulation - 

• HLHS
• Coarctation of aorta .
• interrupted  aortic arch

• Mixed   - TGA

4.Hemodynamic classification of PDA  - 

• SILENT - detected by ECHO only
• SMALL -  Q p / Qs   < 1.5
• MODERATE  -Q p / Qs  =1.5 - 2-2
• LARGE - Q p / Qs > 2.2
• PDA  WITH   EMS

VSD

1 . Aortic regurgitation seen in what % of cases in VSD -

•  5%

2 . Which coronary cusp prolapse in VSD -

•  Infundibular VSD -  Right coronary cusp

•   membranous VSD  - Non coronary cusp 

3 . what % of cases in VSD infundibular stenosis develops - 

• 5%

4.incidence of Infective Endocarditis in VSD 

•  0.2%per year 

5. Best angiographic view to visualise VSD  - 

• LAO 45  , 30  CRANIAL

CLINICAL INFORMATIONS

1. Duration of cyanosis after which clubbing can appear - 

-  6 months

2. opening snap can be present commonly -

• - MS.
• - TS
• - Ebsteins Anomaly

3 . Cyanotic congenital heart diseases with LV apex-

• Tricuspid atresia
• PS with intact IVS 
• Univentricular heart ( LV morphology)

 4. In a newborn  with VSD  when  physiological fall in PVR  not seen  - 

• when VSD is large and size  > 1 sq. cm/  sq m of  BSA

5 . Ductus arteriosus develops from which arch - 

•  6 th arch

6. LV Volume overload

• In  v5 /v6 

• Deep q
• Tall R 
• Peak T

ATRIAL SEPTAL DEFECT

1 . ASD patients become symptomatic by age –

-        4^th   - 5^th decade

2. spontaneous closure of ASD occurs by

   -   33%  -  in 1- 2 years

   -   3%  - in 2-4 years

3.due to chances of spontaneous closure  elective repair of ASD can be delayed  upto –

  - 4 years

4. wide and fixed split of   S -2  in ASD is due  to –

     - FIXED SPLIT  due to-  constant volume of blood in RA  in different phases of respiration which passes through  PA.

 this happens due to  -

reciprocal changes in IVC –to – RA  shunt and  Left to right shunt through  ASD  during respiratory cycles 

-        WIDE SPLIT  - is due to prolong emptying of RV  - due to –

§  Increased RV volume  due to shunt

§  Dilated PA with increased   HANGOUT INTERVAL

5. Type of ASD associated with  most commonly associated with  PAPVC  -

-        sinus venosus type of ASD