Abnormal Pulmonary function, EKG Pectus excavatum

[lizabet61]

17/F , height 69 inches, weight 125 lbs, mild mannered child on no medications medical history very infrequent cold, sore throat,headache. c/o Fatigue, SOB, Chest tightness, suprasternal flutters/ tickly feeling upper chest, hard to take a deep breath, Pounding pulse, tachycardia to 140's when getting up from sitting or lying position. with a BP of 135/90 (once 210/126 with c/o extreme pounding of heart) Mild Pectus excavatum. Chest x-ray mild pectus excavatum with medially displaced vessels, no pneumonia. EKG- Normal sinus rhythm with RSR or QR pattern in V1 sugessitive of right ventricular conduction delay, possible left atrial enlargement. PFT- FVC (L) 3.53 (81%)/ FEV1 (L) 3.17 (84%)/ FEV1/FVC(%) 90 (103%)/ FEF 25-75% 4.95 (120%)/FEF Max 6.45 (86%)/MVV 84 (67%)/ TLC 4.09 (71%) / SVC 2.39 (55%)/ IC 1.28 (49%)/DLCOune(ml/min/mmHg) 23.28 (75%)/ Raw (cmH20/L/s) 104 (56%)/ Graw (L/s/cmH2O)1.05 (102%)/ sGaw(l/cmH2o*s) 0.42 (210%). episodes of this heart racing/pounding frequently daily with position change baseline BP 100/60/, resting Heart rate 76, resting resp rate 14. Help

[John Kenyon, CNA]

Hello -

At baseline this set of complaints is far more common in tall, thin young adults, especially with pectus excavatum. What I'm wondering is if the rapid rate on standing is measured per standard TILTs, after one minute standing, or if it is seen immediately upon standing. There is a big difference between the two that's underappreciated.

There are any number of relatively benign possible causes for this presentation, from "variant of normal" to MVP syndrome to Marfan syndrome, with many in between. Again, much of this may be WNL for the patient given height/weight and PEx.

Resting rate and BP are absolutely normal, so orthostasis is the first and most likely culprit, but echocardiogram might be useful to rule out some of the more bothersome possible disorders which could be suggested by the complaints.

While none of this would seem to require urgent evaluation, I would suggest at least a timely cardiological workup. PFTs are very good. It would be wise to rule out any structural heart abnormalities, if this has not already been done.

I hope this is helpful. Best of luck to you both and please follow up here with us as needed.

[lizabet61]

We have am appointment for ECHO soon. The tachycardia seems to happen immediately on rising and decreases rapidly. She does have a very thin frame, however she has stretch marks on hips and knees, as well as very limber joints, long fingers toes, etc. (?Marfan's) I am curious to see if there is a Mitral valve prolapse. They are going to put a monitor on her for a few weeks to see the frequency of tachycardia. It seems brief now, and the highest it has gotten is 140's. It seems to happen so frequently and has progressively happened more frequently, causing dizziness (and her toes turn blue.) I'm just overly concerned I'm sure. Thanks for the encouragement.
'

[John Kenyon, CNA]

Good news about the scheduled echocardiogram. That's probably going to be the most telling thing that's been done so far. Don't be at all surprised if MVP shows up, as it is very common anyway, more common in females, and especially so in those who may have markers for Marfan syndrome. Your daughter has a few, by the way, but a doctor can actually measure the length of the arms and legs to see if they're disproportionately long, etc. There are a couple other genetically transmitted things like Marfan's which also can be checked for.

The fact that the heart rate accellerates a lot upon standing isn't necessarily a bad thing, and over time many people adapt to this. It often occurs among tall, thin adolescents, but it's also worth noting in the context of everything else. The toes turning blue is called acrocyanosis, and usually isn't meaningful.

Please don't feel you're overly concerned. This is your child we're talking about. This is important stuff. It will be good for both of you to know what is (or isn't ) going on. You're being a good, concerned parent.

Thanks for the follow up and we'll be looking forward to more when you learn it. Best of luck to you both.

[lizabet61]

Saw the pediatric cardiologist today. Absolutely normal ECHO and repeat EKG unchanged, still a conduction delay ( RBBB she said is normal for 17 year olds), otherwise normal heart I am very relieved. Now all we have to do is work on Katelyn's Lung function. Mild airway restriction isn't bad. We see a Pectus excavatum specialist end of January, but I am confident she will not need corrective surgery. Maybe this whole thing is due to the growth spurt she just experienced. I am just thankful her heart is fine! Thank you for keeping me calm.

[John Kenyon, CNA]

Great news. Thanks so much for this update. It's true RBBB is seen, for some reason, more often in adolescents, then again in later middle age, but usually has no significance in either group. It seems you have things pretty well under control at this point, which is really nice to hear. I'm also happy if my input was helpful in keeping you calm in the interim. Working with lung function will be aided by seeing the PEx specialist, I'm sure, and yes, a growth spurt could have set off this whole chain of events. I'm just gratified that everything's looking good. Please keep us updated. Good luck to you both.

[AKzzz]

Hello everybody! I live in France, i'm training in kick boxing since 2 years.

I have a pectus excavatum and i need responses about it:

- can i practice kick boxing with PE?

- With musculation, it possible to dig it ? and with years it is possible to have
difficulty breathing and what age ?
- After Operation NUSS or RAVITCH, it is possible to practice KIck boxing ?

Thanks to give answer :) i need it really