Secondary hyperlipidemia - Causes

From Pediatrics 2011;128:S213-S256

Causes of Secondary Hyperlipidemia

(i) Exogenous:

   Alcohol

   Drug therapy: Steroids, Isotretinoin, Beta-blockers, some OCPs, select chemotherapy agents, select anti-retroviral agents.

(ii) Endocrine/metabolic:

   Hypothyroidism/Hypopituitarism

   Type 1 and 2, DM

   Pregnancy

   Polycystic ovarian syndrome

   Lipodystrophy

   Acute intermittent porphyria

(iii) Renal:

   Chronic renal disease

   HUS

   Nephrotic syndrome

(iv) Infectious:

   Acute viral/bacterial infections

   HIV

   Hepatitis

(v) Hepatic:

   Obstructive liver disease

   Biliary cirrhosis 

   Alagile syndrome

(vi) Inflammatory diseases:

   SLE

   JRA

(vii) Storage diseases:

   Glycogen Storage disease

   Gaucher disease

   Cystine-storage disease

   Juvenile Tay-Sachs disease

   Nieman-Pick disease

(viii) Other causes:

   Kawasaki

   Anorexia nervosa

   Post-solid organ transplant

   Childhood cancer survivors

   Progeria

   Idiopathic hypercalcemia

   Klinefelter syndrome

   Werner syndrome

Lipid - Elevated Triglyceride Treatment Algorithm

From Pediatrics 2011;128:S213-S256

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Lipid - Elevated LDL treatment algorithm

From Pediatrics 2011;128:S213-S256.

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Lipid Levels - Cut-off Values

From Pediatrics;2011:128:S213-S256.

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Ectopy: Atrial vs. Ventricular?

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Atrial ectopy with aberrant conduction. Note: Small P wave is noticeable at the end of T wave preceding the ectopies.

EKG Quiz: Pacemaker

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Assuming the pacemaker is working appropriately, guess the pacemaker mode and set-rate.

Comparison of atrial rate to paced rate holds a clue to the answer.

What could be the indication for pacemaker?

What are the reasons for a 17-yr old to have this mode of pacing?

Heart Rate Variability Analysis - A primer

Link to a primer on this subject.
http://www.macses.ucsf.edu/research/allostatic/heartrate.php

Nutrition: Idaho Plate Method (CDC 2007)

Link to Idaho Plate Method from CDC:

Food Pyramid Transformation

June 2011 - ChooseMyPlate Campaign

2012 We Can campaign sponsored by NIH
(Ways to Enhance Children's Activity &Nutrition)

2010 Dietary Guidelines Link

2007 CDC - Idaho Plate Method

2005 Guidelines

DASH Eating Plan
(Dietary Approaches to Stop Hypertension) - NIH ?1999
PDF handout for parents from NIH
Standby for details on DASH...

1992 Guidelines

Physical Activity Guidelines for Americans 2008

Published by HHS.
Link to a PDF version of booklet.
Or, visit http:/www.health.gov/paguidelines

How to Read Food Labels

Learning how to read food labels is an important step, to teach patients and families.
Link to AHA website (Food label page):

Guidelines for reduction of CV risk for children

AAP Guidelines - Nov 2011. (Pediatrics 2011;128 (Suppl 5): S213). Published in print Dec 2011.
This provides guidelines for management of multiple cardiovascular risk factors in children including - Weight, Diet, Lipd disorders and Hypertension.

Cardiovascular Health and Risk Reduction in Children - NHLBI & HHS publication October 2012 (Booklet - PDF version, 89 pages).

These two guidelines are the same!

Dietary Guidelines for Americans 2010

This is an useful reference - also included guidelines from children. Click here for a PDF version
Or visit the website: www.dietaryguidelines.gov

Choose My Plate compaign (Jun 2011): http://www.choosemyplate.gov/

Nutrition: Salty Six

According to American Heart Association:
The "Salty Six"
1) Breads & Rolls (230 mg per piece)
2) Cold cuts and cured meat (1 g per pre-packaged turkey)
3) Pizza (760 mg per slice)
4) Chicken (amount depends on type of processing)
5) Soup (940 mg per cup)
6) Sandwiches (Ketchup, mustard,..) (1.5 g per sandwich)

Problem with this list is that it is not memorable! And, the items in the list are not mutually exclusive. For example, a sandwich contains breads/rolls, cold cut/cured meat or chicken. Thus, 1, 2 and 4 are actually the 6!

Angiotensin receptor blocker (ARB) in Marfan syndrome

NEJM 2008;358:2787-95.

Losartan started at 0.6 mg/kg/day x 3 weeks. Increased gradually to 1.4 mg/kg/day.
Ibesartan started at 1.4 mg/kg/day. Increased to 2.0 mg/kg/day

Link to posting about this article in Journal Club blog.

MRI Safety & Devices

Website with information regarding safety of MRI in patients with implanted devices.
http://mrisafety.com/

Incomplete Kawasaki - Algorithm

Algorithm for diagnosis of Incomplete Kawasaki Disease

(Circulation 2004;110:2747-2771)

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Consensus Statement on Interpretation of Troponin Elevation

Link

Coming soon in JACC 2012;60.
See video in ACC website

Aortic Root Normal Values - Nomogram

Normal values derived from 353 children (Paris)

AJC 2010;105:888-94.

Different from other nomogram by Roman MJ, et al. AJC 1989. Also available in this blog. 



Boys



Girls



Solid line - Boys
Dotted line - Girls

Indication for Surgery in Marfan Syndrome

Single institution experience. Take it with "a pinch of salt".
(Mt Sinai Medical Center, New York, NY - Dr. Randall Griepp is the senior author)

Diameter = Diameter at Sinus of Valsalva (SoV)
Ratio (Aortic ratio) = Observed diameter/Predicted diameter at SoV

Predicted diameter of SoV is determined from regression formula ("reportedly" derived from Roman MJ et al. AJC 1989:64:507-12). It "appears" that the regression formulas dervice the expected mean value - not clarified in the referenced article (Leget et al. Heart 1996;75:389-95).
Regression formula for "Predicted diameter of SoV" in cm. are as follows:
Child (< 18 yrs): 1.02 + (0.98 x BSA in m2)
Adult (18-40 yrs): 0.97 + (1.12 x BSA in m2)
Adult (> 40 yrs): 1.92 + (0.74 x BSA in m2)

Ann Thoracic Surg 1999;67:1834-9.

Prosthetic Valve - Valve Orifice Area

For St. Judes Valve: (From Manufacturer's website)

Useful to evaluate for Patient-Prosthesis Mismatch

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DORV classification

DORV types & distribution from a study of 70 specimens.

(From Moss and Adams)

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EKG: Junctional Rhythm with Retrograde P waves...

The EKG shows Junctional Rhythm with retrograde P wave, but only in alternate beats.

There appears to be a small P before the junctional beats that do not have a retrograde P wave!

What is the explanation for such phenomenon?

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Cath: Double Balloon Size Selection

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(1) Dr. Rao's book p. 49 (Wiley-Liss, New York, 1993)

(2) Table is from Dr. Jim Lock's book 2nd ed. p. 154 (Kluwer Academic Publishers, Boston 2000)

 (3) Further simplified formula:  (Narang R, et al. Cardiology 1997;88:271-276)
Effective Balloon Diameter = 0.82 (D1 + D2)

EKG: Pulmonary Embolism

17 yr old girl, admitted with shortness of breath. CT scan showed multiple pulmonary emboli.

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EKG: Intermittent WPW

Intermittently manifest accessory pathway

Note: Two beats in this rhythm strip have normal PR interval without Delta wave.

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Echo: Tissue Doppler Imaging of MV

(Extracted from JASE 2010;23:465-95)

3 cursor locations for AV valves:

Lateral Mitral Annular Tissue Doppler Tracing:

s' - Ventricular Systole

e' - Ventricular recoil during early diastole

a' - Atrial systole

IVCT' - Isovolumic contraction time (Note: Apostrophe)

IVRT - Isovolumic relaxation time (Note: Apostrophe)

Echo: 3D Mitral Valve (s/p AVSD repair, MR)

SVT from Holter - Onset and Termination

An ectopy initiates the re-entry circuit.

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EKG

Right axis deviation, Tall R waves in right precordial leads, Strain pattern (ST depression & T wave inversion) in the leads showing tall R waves. This is RVH with pressure overload (as opposed to volume overload that will lack the strain pattern).

Click here for the source: ECG of the Month at Heart.Org. Posted by Dr. Kyuhyun Wang. June 2012.

Pleural effusion after Fontan Operation

Am J Cardiol 2005;96:130-3
Millwaukee. 2000-2005, n=25 (on protocol) vs. Historic controls from 1997-2000 (n=33). Median duration of pleural effusion decreased (6 days vs. 15 days).

Protocol:
80% maintenance fluids
Lasix 1 mg/kg/dose Q8 hrs, changed to Diuril + Spironolactone Q12 hrs when PO intake starts
Captopril when PO intake starts
Nasal cannula oxygen, at least 0.5 LPM
Low fat diet (30% calories from fat)

(Nitric oxide/Sildenfil)

Other papers:
Curr Opin Pulm Med 2010;16:392-6 (by E. Austin III) - summarizes several recent studies. No signficant conclusions. Highlights the uncertainty of etiology and aproach.

RACHS Score categories

Jenkins, KJ et al. JTCVS 2002;123:110-8

Risk Adjusted Congenital Heart Surgery Score

RACHS category 1:

1. Secundum ASD

2. Aortopexy

3. PDA (> 30 days of age)

4. Coarctation (> 30 days of age)

5. PAPVR repair



RACHS category 2:

1. Aortic valvuloplasty (> 30 days of age)

2. SubAS resection

3. Pulmonary valvuloplasty or replacement

4. RV infundibulectomy

5. RVOT augmentation

6. Coronary fistula repair

7. ASD & VSD repair

8. Primum ASD repair

9. VSD repair

10. Tetralogy repair

11. VSD closure with PA band removal

12. Repair of unspecified septal defect

13. TAPVR repair (> 30 days of age)

14. Glenn shunt

15. Vascular ring surgery

16. A-P window repair

17. Coarctation repair (≤ 30 days of age)

18. PA stenosis repair

19. Common atrium closure

20. LV-RA shunt repair



RACHS category 3:

1. AVR

2. Ross procedure

3. LVOT patch

4. Ventriculomyotomy

5. Aortoplasty

6. Mitral valvuloplasty or replacement

7. Tricuspid valvuloplasty or valvectomy or replacement

8. Tricuspid valve repositioning (Ebstein’s) (> 30 days of age)

9. Anomalous coronary artery repair with or without intrapulmonary tunnel (Takeuchi)

10. Closure of semilunar valve (aortic or pulmonary valve)

11. RV-PA conduit

12. LV-PA conduit

13. DORV repair with or without RV obstruction

14. Fontan

15. AVSD (complete or transitional) repair with or without valve replacement

16. PA banding

17. Tetralogy with Pulm. Atresia repair

18. Cor triatriatum repair

19. Systemic-Pulmonary artery shunt

20. Atrial switch operation

21. Arterial switch operation

22. Pulmonary artery reimplantation

23. Annuloplasty

24. Coarctation & VSD repair

25. Cardiac tumor excision



RACHS category 4:

1. Aortic valvuloplasty (≤ 30 days of age)

2. Konno procedure

3. Complex defect (Single ventricle) repair with VSD enlargement

4. TAPVR repair (≤ 30 days of age)

5. Rastelli procedure

6. Atrial switch with VSD closure

7. Atrial switch with subpulmonary stenosis repair

8. Arterial switch with PA band removal

9. Arterial switch with VSD closure

10. Arterial switch with subpulmonary stenosis repair

11. Truncus repair

12. Repair of … or interrupted aortic arch with or without VSD repair

13. Unifocalization … Tetralogy-PA

14. …



RACHS category 5:

1. Tricuspid valve repositioning for neonatal Ebstein’s (≤ 30 days of age)

2. Truncus with Interrupted aortic arch repair



RACHS category 6:

1. Norwood operation

2. …

Narrow Complex Tachycardia - Atrial Flutter

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Anatomy: Interrupted IVC with Azygos Vein Continuation

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Link to Azygos Vein anatomy images

Pacemaker - Non-capture

Attempt to detect the underlying rhythm. (Postop. patient)
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Differential Cyanosis (& Clubbing)

21 yr old man with Unrepaired PDA and Severe Pulmonary hypertension.

Cyanosis and Clubbing are present in the feet.

No cyanosis or clubbing in the hands.

(Both feet were affected by post-polio paralysis as a young child and now, wheel chair bound)

Retroaortic Innominate Vein

Panel A: (6th week of gestation). 1 & 2 - Right and Left anterior cardinal veins, 3 - Primitive aorta, 4 & 5 - Superior and Inferior transverse venous plexus, 6 - Right & Left common cardinal veins, 7 & 8 - Right and left posterior cardinal veins, 9 - Sinus venosus, 10 - Vitelline vein, 11 - Umbilical vein, 12 - Developing IVC

Konstantinov I. E. et al.; Ann Thorac Surg 2003;75:1014-1016