Lung Ultrasound to Evaluate Pulmonary Edema

 

Prospective clinical study of 44 newborns, mean body weight 3 kg. All with critical congenital heart diseases and underwent heart surgery. CXR and Lung US were compared to guide management of diuretics, vasoactive inotropes, etc. Good correlation was noted.

An element of subjective assessment is needed. Probably, may not replace CXR. Or, it will take a very long time to replace CXR as an exclusive tool to assess pulmonary edema.

Basak Kaya et al. Pediatrics & Neonatology 2024 xxx (xxx) xxx.

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.

European Guidance for screening for Sudden Cardiac Death in athletes

 European Journal of Preventive Cardiology 2015

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

Thoracic duct redirection to left atrium - Fontan