Friday, October 6, 2023

Dysautonomia and Pregnancy

 Short article in Dysautonomia International blog page: Link to the article

By Dr. Svetlana Blitshteyn

Guest author Svetlana Blitshteyn, MD is the Director of the Dysautonomia Clinic and a Clinical Assistant Professor of Neurology at the University at Buffalo School of Medicine and Biomedical Sciences.

Excerpts:

  • POTS patients may have associated gynecologic issues such as endometriosis and fibroids. Therefore, needs careful evaluation by gynecologist, POTS physician and patient.
  • During pregnancy, 60% women with POTS reported severe hyperemesis gravidarum and 40% fatigue. Higher rate of miscarriage is reported.
  • During pregnancy, 30-50% women reported worsening of POTS symptoms. (50% had no change in their POTS symptoms.
  • After delivery, 50% of women reported improved symptoms for 6 months after delivery. In a different study, 30% reported worsening symptoms after delivery. 70% reported stable symptoms after delivery.
  • POTS medicines during pregnancy:
    • There are no POTS meds in Class A list by FDA (these are considered safe during pregnancy).
    • I have used low-dose beta-blocker during pregnancy to control tachycardia
    • Florinef and Pyridostigmine (Mestinon) are continued during pregnancy if necessary.
    • Less experience with Midodrine (its newer).
    • Women on SSRI's may switch to Prozac.
    • Medications that need to be weaned off or used sparingly include Benzodiazepines (e.g. Clonazepam, Ativan), Xanax (Alprazolam) and stimulants (Ritalin, Adderall).
  • POTS Medicines, to be stopped during pregnancy
    • Ideally, stop all medicines prior to conception. But, this may be unrealistic.
    • Planned pregnancy: 1st trimester - stop meds or reduce dose to minimum. If planning a pregnancy, slowly wean benzodiazepines and stimulants.
    • Unplanned pregnancy: wean above meds on a faster schedule.
  • Risk to the baby (in utero or postpartum)
    • 4 studies - no negative effects.
    • Does fainting harm the baby? No
    • But, recurrent syncope may reduce placental blood flow and therefore, may need an active management.
  • Breastfeeding
    • Metoprolol is safer than atenolol.
    • Prozac which is acceptable during pregnancy may be more harmful to the baby via breastmilk (Zoloft is better).
  • Will the child develop POTS?
    • Not enough studies to answer this question. 
    • Familial incidence occurs in 13-40% of patients.
References:
1. Blitshteyn S, Bett GL, Poya H. Pregnancy in Postural Tachycardia Syndrome: clinical course and maternal and fetal outcomes. J Matern Fetal Neonatal Med. 2012; 25: 1631-1634.

2. Kanjwal KK, Karabin B, Grubb BP. Outcomes of pregnancy in patients with Postural Orthostatic Tachycardia Syndrome. PACE 2009; 32:1000-1003.

3. Peggs, KJ, Nguen H, Enayat D, et al., Gynecologic disorders and menstrual cycle lightheadedness in postural tachycardia syndrome. Int J Gynaecol Obstet. 2012; 118: 242-246.

4. Kimpinski K, Iodice V, Sandroni P, Low PA. Effect of pregnancy on Postural Tachycardia Syndrome. Mayo Clin Proc 2010; 85: 639-644.

5. Powless CA, Harms RW, Watson WJ. Postural tachycardia syndrome complicating pregnancy. J Matern Fetal Neonatal Med 2010; 23: 850-853.

Monday, May 9, 2022

IV Sotalol - Registry study in children

 85 patients

Age range 1 day - 36 yrs)

Treated with IV Sotalol (Average dose 1 mg/kg/dose; Range = 0.5 - 1.8 mg/kg/dose).

Infused 

over a median period of 60 min (Range 30 min - 5 hours).

Successful in 49%, Improved in 30% (Improved = HR decreased to the extent of allowing overdrive atrial pacing).

QTc prolonged >465 ms in 16% (Prolonged >500 ms in 4%).

Conlcusion: Safe as effective.

Successful or Improved in 79%.

Most common dose is 1 mg/kg/dose, infused over 60 min.

Link to reference: Mollory-walton LE at al. JAHA 2022. 

Rivaroxaban (Xarelto, Janssen Pharmaceuticals, Inc)

 FDA approved for 2 pediatric indications:

1) Venous thromboembolism in children - after at least 5 days of parenteral anticoagulant treatment.

2) Thromboprophylaxis in pediatric patients < 2 yrs of age after Fontan operation.


Link to manufacturer information page and IFU.

Mavacamten for HOCM

 FDA approved Mavacamten (Camzyos from Bristol-Myers Squibb) for HOCM for children (May 2022).

Mavacamten is allosteric inhibitor of Myosin


Explorer-HCM trial data showed 

    (i) improved in peak VO2 and 

    (ii) stabilization or improvement in NYHA function class

compared to placebo.


Long-term extension study showed that benefits lasted at 1-year follow up. There was improvement in QoL reported by patients.


Valor-HCM - addition of Mavacamten to maximally-tolerated medical management, reduced the need for surgical or cath intervention for septal reduction.


News from TCTMD dt. 4/29/22


Sunday, August 28, 2016

High-Risk Cardiac Disease in Pregnancy

Uri Elkayam, Sorel Goland, Petronella Pieper and Candice Silverside.
(UCLA, Jeruselam, Groningen - Netherlands & Toronto)

Good review articles covering most important lesions.

Part I - JACC 2016;68(4): 396-410.
Part II - JACC 2016;68(5): 502-16.