Saturday, July 26, 2025

How late can AV block develop in children of mothers with SSA antibodies?

 Makadia L, et al. Am J Perinatology Reports 2023;13:e29-e34.

Pubmed search: 2 groups of studies

Group 1 - Prospective studies (Prenatal)

Group 2 - Retrospective studies.


Group 1: 10 studies, reporting 1248 cases.

Group 2: 9 studies.


Take home points:

From the Introduction:

Maternal autoantibodies cross placenta in 2nd trimester and cause inflammation that leads to AV block. Therefore, screening should start at 16 weeks gestation.

Maternal autoantibodies clear infant's blood by 6 months of age. Therefore, usual onset of AV block is within first year.


From a Swedish Congenital Heart Block Study Group - 1949 - 2009. 190 cases were reported. However, the values in the abstract or in Table 2 of the Makadia et al. report do not correlate. Nevertheless, there were 9 patients diagnosed in the age 4 mo - 18 yrs and 4 patients diagnosed in the age > 18 yrs. (Need to reconcile the numbers with original publication). Bergman G et al. Acta Paediatr 2014;103:275-81.

Friday, August 9, 2024

Terminology of names of targeted bio-therapies

 The Names of Targeted Therapies Give Clues to How They Work | ONS Voice

The passage below was cut and pasted from the abovementioned article: 

Very helpful

Presently, two main families of targeted therapies exist—monoclonal antibodies and small molecule inhibitors. The ending letters (stem) of the generic names are like surnames that tell what family the drug is from and how the drug works to kill cancer cells. Monoclonal antibodies end with the stem “-mab” and small molecule inhibitors end with the stem “-ib”. The “-mab” family of targeted therapies has three distinct methods for interfering with cancer cell growth.

  1. Attach to receptors on the outside of cells to prevent the receptors from interacting with signaling molecules (e.g., growth factor receptors and growth factor interaction)
  2. Deliver radioactive molecules or toxins to the inside of the cells through attachment to cellular receptors
  3. Activate the body’s natural immune response

The “-mab” family is used when receptor targets are overexpressed on the outside of cancer cells. Conversly, the “-ib” family targets processes within the cell and therefore must be small enough in molecular weight to enter the cell and interfere with proteins on both the inside and outside of the cell. Proteins that code for growth or inhibit growth are some of the targets of these small but powerful family of drugs.

The sub stem of the generic names of the “-mabs” identifies the source where the antibodies were generated or cloned. The three most common sources are

  1. Chimeric human-mouse—drugs ending in “-ximab” (i.e., rituximab)
  2. Humanized mouse—drugs ending in “-zumab” (i.e., bevacizumab)
  3. Fully human—drugs ending in “-mumab” (i.e., ipilimumab).

Finally, both “-mabs” and “-ibs” contain an additional stem to describe the targeted therapies bullseye. For example, the “tu” in rituximab indicates the target is the tumor, the “ci” in bevacizumab designates the circulatory system, and the “li” in ipilimumab identifies the immune system target. Some of the intracellular targets for the “-ibs” include:

  1. Tyrosine kinase inhibition—sub stem “-tinib” (i.e., imatinib)
  2. Proteasome inhibition—“-zomib” (i.e., bortezomib)
  3. Clyclin-dependent kinase inhibition—“-ciclib” (i.e., seliciclib)

The prefix of the generic names and the drug market names are where researchers and pharmaceutical companies—like the parents of the young dancers—take creative liberty.  

The development of targeted therapies is expected to accelerate as new targets are identified, as a result, oncology nurses will need to stay up to date on the new medications so they can educate their patients on the way these therapies work as well as the possible side effects of the medications. Unfortunately, many people may have the idea that there are few if any side effects associated with targeted therapy. Although side effects can be less than those of standard chemotherapy, targeted therapies also affect normal cells to some degree.

So how do nurses keep up on the new therapies? Recently, ONS merged two key special interest groups (SIGs) the Targeted and Biologic Therapies SIG and the Chemotherapy SIG. The new SIG is now called the Chemotherapy and Biotherapy SIG. Another resource I found especially helpful was an animated video titled Understanding Targeted Cancer Therapies presented by the National Institute of Health.

Friday, March 22, 2024

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.

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.

Friday, August 26, 2016

Late Causes of Death After Pediatric Cardiac Surgery

Alireza Raissadati et al. JACC 2016;68:487-98.
From Finland.

1953-2009
Divided into two surgical era: 1953-89 & 1990-2009.

Cardiac causes of mortality:
Heart failure
Pulmonary hypertension
Sudden death (Aortic rupture, Arrhythmia, MI - majority were unexplained)
Death after reoperation

Non-cardiac causes of mortality:
Respiratory disease (esp. pneumonia associated with mental disability)
Neurologic and infectious diseases
Neoplasm (more common compared to general population)
Accidents (alcohol-related)

Saturday, May 28, 2016

Tuesday, November 17, 2015

Intraluminal PA band - Surgically placed, Balloon dilatable.

Feasibility and related outcome of intraluminal pulmonary artery banding

Stany Sandrio et al. EJCTS 2015;48:470-480.

Pericardial patch with a fenestration is placed inside MPA. Needs cardiopulmonary bypass to perform this surgery.
Two types: One patch with a fenestration, or Two patches sewn together at the fenestration and then, sutured inside MPA to create an hour-glass appearance.

Balloon dilatation of this patch is possible.
Significant number of patients needed ECMO support after the procedure.
Total patients 32 in the study.



Trusler rule: JTCVS 1984;88(5 Pt 1): 645-53.

Sunday, October 11, 2015

Evrolimus therapy for Rhabdomyoma

From Wagner R. et al. Echocardiography 2015. Online accessed September 2015. DOI:10.1111/echo.1 3015.

Newborn. Treated with Evrolimus (1 mg/m2 once daily). Resolution after 3 weeks of treatment. Trough level of Evrolimus was monitored. Lymphopenia and hypertriglyceridemia was noted when higher dose was used for the first 2 days.

Sunday, September 13, 2015

Congenital heart defects and receipt of special education services

Tiffany Riehle-Colarusso et al.
Pediatrics 2015;136:496-503.

Compared with children without birth defects, children with CHD were 50% more likely to receive special education services (Adjusted Prevalence Rate Ratio 1.5)

Special education categories:
Intellectual disability (aPRR 3.8)
Sensory impairment (aPRR 3.0)
Other health impairment (aPRR 2.8)
Significant developmental delay (aPRR 1.9)
Specific learning disability (1.4)

Saturday, August 22, 2015

Sildenafil - liquid preparation

Click on the image to enlarge
(10 mg/ml)


New therapy for high LDL - PCSK9 inhibitor

From an advertisement by AmGen in Cardiology News August 2015:
New therapy for high LDL level.
PCSK9 inhibitor
PCSK9 (Proprotein convertase subtilisin/kexin type 9) degrades LDL receptors in hepatocytes.
Injection Praluent (Alirocumab) 75 mg or 150 mg. self-administer, q2weeks, recently approved by FDA.



Hyperkalemia therapy - Patriomer

From Cardiology News Aug 2015
Patiromer - a pottasium binding polymer. Binds with K in GI tract and enables excretion in stool.
JAMA 2015:314:129-30.

New Heart Failure Medication - Sacubitril (Neprilysin inhibitor)

From Cardiology News Aug 2015:
RE: FDA approval for a combination medication (Sacubitril + Valsartan)
Sacubitril - a neprilysin inhibitor
Valsartaion - an angiotensin receptor blocker