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)

Pulsus Paradoxus

How to meaure it?

(Click on image to enlarge)

You need a manual sphygmomanometer.

We are looking for decrease in systolic BP between inspiration and expiration.

First, determine the BP of the patient. Then, raise the pressure in the cuff above the measured systolic pressure and bring it down slowly (2-5 mmHg steps) - holding the cuff pressure at each level for at least 2 breaths. When Koratkow sounds are first heard (take this as expiratory systolic BP), these will be intermittent because these sounds are heard only in expiration and not heard during inspiration. Thus, in the initial part of the descent, you only hear Koratkow sounds intermittently. (First arrow in the figure above)

Continue to decrease the cuff BP (slowly) - holding at each step for at least 2 breaths. There will be point when Koratkow sounds are heard continuously. In other words, you would suddenly hear more Koratkow sounds than before. This is the inspiratory - systolic BP (Second, lower arrow in the figure above).

Difference between expiratory systolic BP and inspiratory systolic BP should be < 10 mmHg. If higher (20 or above), pulsus paradoxus is supposed to be present. Pulsus paradoxus is the exaggeration of a normal phenomenon.

Pulse ox trace and Arterial line trace - if available, are also useful to recognize pulsus paradoxus.

Other signs of pericardial tamponade (or restriction) is Kussmaul sign. This is inspiratory increase in JVP.  This is what is opposite of normal. Apparently, some how, the term paradoxus has transferred to pulsus paradoxus?!

(Figure from Cardiovascular Dynamics by Robert F. Rushmer, 3rd ed. 1970 W.B.Saunders Company, Philadelphia.)

Newborn with Congenital Heart Disease - Clinical Approach

Algorithm for diagnosis of congenital heart disease in newborn

Click on the image to enlarge

Sorry, can't remember the source of this flow chart.

Echo features of tamponade

14 yr old with history of intermittent facial swelling (SVC syndrome). Superior mediastinal widening and cardiomegaly on CXR.





Diastolic collapse of RV free wall.


Doppler equivalent of pulsus paradoxus (?!)

Echo: Cardiac Tamponade Video

Link to 2 nice videos

https://www.stanford.edu/group/ccm_echocardio/cgi-bin/mediawiki/index.php/Tamponade

ICU: Monitor - Differential Cyanosis

Scenario 1:
6-hours old, term baby girl. 3.4 kg. Transferred from outside hospital for cyanosis noted at 2 hrs of age.
Oxygen saturation:
Preductal 100%
Postductal 81%

Scenaio 2:

17 day old baby, born at home

Admitted via ER for cyanosis and respiratory distress.

Following is the monitor image:

"Reverse" Differential cyanosis is noted

Preductal saturation 87%

Postductal saturation 96%

What is the differential diagnosis?

Cath: Screening before PFO closure

Name, Age, Gender

Handedness

Race

PMH: CAD, DM, HT, Hyperchol, Afib, Carotid Dz, PE, DVT, First trimester miscarriage (How many), Migraine (with or without aura)

Family Hx: PE, DVT, Miscarriage

Smoker: Current, Former

ETOH: (amt, current, Former)

Meds at the time of episode: (Antiplatelet, Warfarin, Statin, OCP, Other)

Initial complaint: (Description, Date & Time)

Nature of episode: TIA, Stroke, Peripheral embolism, Other, Retinal ischemia

Valsalva in history: (lifting, coughing, straining, laughing, sex within 30 min of onset). If yes, explain

Prolonged immobility: (hospitalization, travel, etc. within 1 week of episode)

Concurrent illness:

Previous spell suggestive of TIA/Stroke/Peripheral emb - Dates if yes)

Evidence of multiple lesions on exam

Cardiac murmur

Crochetage in EKG

Investigations:

Protein C & S

AT-III

Factor V Leiden (APC res.)

Lupus anticoagulant

Anticardiolipin AB

Antiphosphotidylserine Ab

Prothrombin gene mutation

Homocysteine

Cholestrol panel (TC, HDL, LDL, TG)

TOAST criteria:

Large vessel (Probable, Possible)

Cardioembolic (Probable - Lesion _____, Possible)

Some references:

JACC 2005;46:1768-76. State of the art paper. Patent Foramen Ovale: Current pathology, pathophysiology, and clinical status.

Stroke 2004;35:803-4. Patent foramen ovale and recurrent stroke: Closure is the best option: Yes.

Stroke 2004;35:804-5. Patent foramen ovale and recurrent stroke: Closure is the best option: No.

Stroke 2004;35:806. Patent foramen ovale and stroke Closure by further randomized trial is required.

Cath: Coronary Fistula

Case #1

Asymptomatic murmur in a 2 year old. H/O Asthma since 6 months of age

(The following images are from Balaguru et al. Catheter Cardiovasc Interv 2006;67:942-6)

Additional reference:

Valente, AM, et al. Circulation Cardiovascular Interventions 2010;3:134-9. Predictors of long-term adverse outcome in patients with congenital coronary fistula (Some what adult oriented study from Boston Children's Hospital. The following image is from this article, showing mural thrombus in mid-circumflex branch - 2 yrs after transcatheter closure).

General: Infective Endocarditis

Decision making re: surgery and its timing in acute infective endocarditis:

References:
1) Eur J Cardiothorac Surg 2009;35:130-5. Hickey EJ, et al. (Toronto group)
Authors report surgical experience of 30 children operated for endocarditis in the past 30 years (1978-2007). Strep. viridans was noted predominantly with known cardiac defects. Staph. aureus was associated with abscess formation, clinical sepsis, acute deterioration and death. Aortic, mitral and tricuspid valves were involved with equal frequency. Pulmonary valve involvement was rare. Native valve was preserved in 22 children (73%). Univariate predictors of valve replacement were increased valve thickening and septic emboli (Severity of valve regurgitation was not predictive). Good paper.

2) Circulation 2010;121:1005-13. Lalani T, et al. Analysis of impact of early Surgery on in-hospital mortality of native valve endocarditis.
Data from an international registry. Non-randomized, retrospective study. So, of limited help. But, still useful information from a large cohort. Conclusion is that early surgery for native valve endocarditis has benefit of lower mortality compared to medical therapy alone. (Defn. of early surgery is replacement or repair of valve during initial hospitalization for IE).
...Read als0, the editorial on this article at Circulation 2010;121:960-2.

3) European guidelines for prevention, diagnosis and treatment of infective endocarditis. Quite useful. Eur Heart J 2009;30:2369-2413.

4) American Heart Association Guidelines:
(i) ACC/AHA 2008 Guideline Update on Valvar Heart Disease: Focus on infective endocarditis. Circulation 2008;118:887-896.
(ii) ACC/AHA 2005 Guideline: "Infective Endocarditis: Diagnosis, Antimicrobial therapy and management of complications" Circulation 2005;111:3167-3184. Or, the full text version (44 pages long) from AHA website.

Pulmonary vein stenosis

Surgery: Sutureless repair of Pulmonary vein stenosis:
(Lacour-Gayet F, et al. Arch Mal Coeur Vaiss 1996;89:633-36)

Modification to Sutureless Repair of Pulmonary vein stenosis (Toronto group):

(Figure from Najm HK, et al. JTCVS 1998;115:468-70Follow this link for pericardial reflections

Additional papers:

Seale AN, et al. Heart 2009;95:1944-9. Pulmonary vein stenosis: the UK, Ireland and Sweden collaborative study. Insightful epidemiologic article about this rare disease.

ALCAPA - Importance of Color Doppler Signal & Unfortunate artifact in 2D

Following 3 images are from a 14 yr old boy complaining of exercise-related dizziness, palpitation and chest discomfort.


Figure 1: Parasternal short axis view...unfortunately has an artifactual drop out which was misleading. Here, LCA appears to be clearly originating from aorta. However, the color Doppler signal in second image raises a red-flag.




Figure 2: This image with color flow Doppler signal in LCA indicates "wrong" direction of flow in LCA. This finding lead to performance of an angiogram (Figure 3).


Figure 3: RAO view of selective left coronary angiogram showing retrograde filling of left coronary system which ultimately drains into Main Pulmonary Artery. Venous catheter is in MPA.

Retrospectively, there is a "drop-out" of the thin membranous separation between Aorta and LCA which is originating from MPA, but travel close to the wall of aorta. This emphasizes the importance of showing coronary origins both by 2D and color Doppler.

Also, such demonstration with 2D and color Doppler will eliminate possibility of mistaking transverse sinus of the pericardium around the great arteries for coronary arteries by 2D. Color Doppler will show fail to demonstrate flow in transverse sinus (folds of pericardium).

Social Issues: Information resources for Families

www.childrenscardiomyopathy.org

(Service by Children's Cardiomyopathy Foundation)

CHDresources.org

(Service provided by California HEARTconnection, an all-volunteer, nonprofit support network)

Will add more such websites in future.

Clinical Signs - Marfan Syndrome

Wrist sign

Thumb sign

Gen: Coronary Artery Anomalies in d-TGA, TOF, l-TGA...

Normal coronary anatomy: (Figures from Braunwald)

Coronary Anomalies in TOF

(Preoperative angiography in infants with tetrad of Fallot. Review of 39 cases. Am J Cardiol. 1981 Jun;47(6):1279-85)

Coronary Anomalies in d-TGA
Figure from Mavroudis's book:

(Ref: Rossi MB, Ho SY, Anderson RH, et al. Ann Thorac Surg 1986;42:573)

Figures from Eugene Braunwald's Atlas (Vol XII):

(TGA & Coronary Patterns - Ursula Sauer & Adrianna C. Gittenberger-deGroot)

1. General rules (observations) about coronary arrangement in TGA:
Four rules:
(a) Three main epicardial branches exist: ("L" for LAD, "CX" for LCX and "R" for RCA)
(b) Coronaries connect exclusively to the aortic sinuses that face the pulmonary orifice
(c) Connections between ostia and branches tend to take the shortest course; and coronaries never cross each other
(d) Positional nomenclature of the aortic sinuses facing the pulmonary orifice cause confusion.
Therefore, the following nomenclature is proposed by Dr. Gittenberger-deGroot.

2. Common Coronary Patterns in d-TGA:

Patterns of Intramural Coronaries in TGA:

Proximal course of coronary arteries in TGA:

Coronary pattern incidence in different types of TGA:

Figure from Aldo Castaneda's book:

Common Coronary Artery arrangements in d-TGA

Anatomy of the coronary arteries in TGA and methods for their transfer in anatomic correction.

(Yacoub, MH. & Radley-Smith, R. Thorax 1978;33:418-424)

(Figure is a view from front of the patient - observer facing the patient).

Total number of patients in the report = 18

a: (n=11) "Normal for TGA" pattern. Origins are at the center of the sinus.

b: (n=2) Single left coronary (Origin is closer to a commissure than in center of the sinus). LCA travels between great arteries

c: (n=1) Coronaries originate from very close to the commissures

d: (n=3) RCA gives off LCx. LAD alone comes off the left posterior sinus

e: (n=1) LCx has separate origin from right posterior sinus; RCA and LAD have a common origin...I believe that either description or the figure has an error in "e".

Figure from Kirklin/Barratt-Boyes Cardiac Surgery:

(Quaegebeur JM. The arterial switch operation. Rationale, results and perspectives. Thesis; Leiden University, The Netherlands; 1986) & (Quaegebeur JM, et al. J Thorac Cardiovasc Surg 1986;92:361)

(Table from Kirklin JW, et al. Circ 1992;86:1501)

Laid-back view to image coronary anatomy in d-TGA

Wernovsky G & Sanders SP. Coronary artery anatomy and transposition of the great arteries. Coronary artery disease 1993;4:148-157

(Original publication: Mandell et al. Am J Cardiol 1990; 65:1379-1383)

Coronary artery anatomy in l-TGA:

Ref: Sequential diagnosis of coronary arterial anatomy in congenitally corrected transposition of great arteries. Ing-Sh Chiu et al. Ann Thorac Surg 2003;75:422-9.

This is an extensive paper. Will review this "soon".