CME INDIA Case Presentation by By Dr Bhanu Pratap Singh, MD, Consultant Physician, Siwan (Bihar).

CME INDIA Case Study

How Presented?

  • 26 yrs. male presented with palpitations and sense of fear for 2-3months duration.
  • Episodic.
  • Clinical examination was unremarkable.
  • Thyroid Profile, Echocardiography, and routine blood tests were normal.
  • ECG – Being displayed.

Dilemma - 26 Yr.-Old-Male with Palpitations and Panic Episodes

CME INDIA Discussion

(03/05/2023)

Many physicians commented like this:

  • ECG normal sinus rhythm.
  • Plan of action.
  • Thyroid Profile.
  • Treat with an Anti-anxiety.
  • Start Anti-anxiety drugs.

ECG was not so simple

Dr. Mahesh Mahajan, Internist, Pimpri Chinchwad, Pune:

  • Short PR interval 100 ms.
  • Causes intermittent palpitations.
  • Do Holter monitor to rule out atrial flutter or tachycardia.

Dr. D. P. Khaitan, Consultant physician, Gaya/Ahmedabad:

  • A mind puzzling ECG, though interesting but found very uncommonly in young patients.
  • Is there availability of recorded ECG with the family members or any history of syncopal episode with the patient or family members? Please let us know.

Dr. Bhanu Pratap Singh:

  • I took the family history as well, but there is no any family history of syncope /SCD or any h/o of syncope to the patient. The only complaint is palpation and sense of fear.

Dr. D. P. Khaitan:

  • There is ECG dictum if a young patient exhibits short QTc (In this ECG, it is 0.35) with either the history of palpitation or syncope episode either with the patient or family member members or the history of unexplained sudden death in the family, one should think in the term of SHORT QT SYNDROME.

Dr. Venkatesh Molio, Maregoan, Goa:

  • Agree.
  • Would keep 2 things in mind short QT And LGL.
  • Short PR also?

Dr. D. P. Khaitan:

  • Yes, I do agree.
  • “PR interval is shortened to 0.10”. There is associated shortened QTc is well. Sometimes such combination might be seen suggestive of dual pathology.

Dr. Bhanu Pratap Singh:

  • Yes, I am thinking of in terms of short qtc (as the patient is symptomatic) along with short PR.
  • Probably an uncommon association. Short QT syndrome is itself less common.
  • It appears, he is a case of short QT syndrome with short PR. Pt is symptomatic, though there is no family history.
  • What next?? ICD? Or observation. Quinidine /Disopyramide?

Dr. D. P. Khaitan:

  • Yes, both conditions with this unfortunate young person with such red signal findings on his ECG.
  • This seems to be the possibility.
  • Please proceed for genotype assessment and consult our cardiologist friends dealing with such cases in their clinical practice.
  • In the meantime, the patient might be put on medical therapy by considering this condition to be sinister in nature.
  • Assess the first-degree relatives by their ECG recording.
  • One query from my side, whether we are dealing with Short QT syndrome as such or with some separate entity dealing with atypical ECG presentation with short PR and QTc interval in the same patient?

Dr. Venkatesh Molio, Maregoan, Goa:

  • Usually, short PR and long QT and sudden death is commonly seen situation.
  • This combination is rare, but seen occasionally.
  • This patient has both and clinical significance in this particular patient needs to be confirmed.

Dr. Mahesh Mahajan, Pimpri Chinchwad, Pune:

  • It’s usually seen in ecgs as heart rate increases, QTc interval decreases.
  • In this case heart is slow 60/min, in spite QTc also decreased, it’s opposite.
  • So, this is Short PR interval with short QTc.
  • There is fast atrial as well as ventricular conduction then pause as bradycardia.
  • Short PR interval 100 ms.
  • Causes intermittent palpitations.
  • Do Holter monitor to rule out atrial flutter or tachycardia.
  • It’s usually seen in ecgs as heart rate increases, QTc interval decreases.
  • In this case heart is slow 60/min, in spite QTc also decreased, it’s opposite.
  • So, this is Short PR interval with short QTc.
  • It is fast atrial as well as ventricular conduction then pause as bradycardia.

Dr. Varun Kumar, DM Card., Sante Vita Hosp., Ranchi:

  • Well, both the conditions which present in this ECG i.e., Short PR & short QT is well discussed.
  • In short PR there is possibility of PSVT which is relatively benign condition but if it is due to short QT then it is due to VT or VF which could be lethal.
  • So, HOLTER monitoring is required to know the cause of syncope.

Dr. Pradeep Sahay, Giridih:

  • The presence of short PR and QTc in the sane individual is rare but restricted to young individuals. Palpitations, sweating and waking up at night are transient symptoms. There is often a family history of sudden cardiac death. This uncommon phenomenon has been described under the heading “Decrease in cardiac electrical systole” by Prof Breijio Marquez in The International Journal of Cardiology, 2007. Many patients are overlooked. A very interesting case.

Dr. D. P. Khaitan:

I wish to elaborate the ECG findings further in the following ways:

  • The QT and QTc interval both are shortened to 0.35″
  • There is hardly and discernible ST segment over precordial leads, with the immediate inscription of Taller T waves.
  • Such T waves are due to acceleration of the final phase of repolarization due to the rapid outpouring of K+ ions in vicinity producing greater electrochemical gradient across the cardiac membrane.
  • The cut point is 360 ms in symptomatic male patient to diagnose SQTS.
  • Now due to presence of additional shortening of PR interval with QT shortening can we think in term of some separate syndrome?
  • This sort of combination is seen most obviously at the heart rate 60 bpm and this is the rate in this ECG.
  • I do agree there that PR interval is 0.10″
  • I wish to draw the attention of the house towards a particular finding on this ECG which can explain the short PR interval associated with SQTS.
  • On careful inspection on this ECG there is depression of the PQ segment in inferior leads and anterolateral leads V5-6 – this indicates a heterogeneous abbreviation of atrial repolarization (compare PQ segment with TP segment).
  • ECG Review: Cardiac channelopathies A simplified approach – compiled by me on Page 54).
  • There is, a higher possibility of change in time dynamic taken from transmission of impulses from SA node to downwards admist the crowding of atrial heterogeneity leading to short PR interval.

Dr. Varun Kumar, DM Card., Sante Vita Hosp., Ranchi:

  • This is one of the rare ECG where u find a combination of short PR and short QT together.
  • This is rare combination has high propensity for malignant arrhythmias & SCD… So, these pts r candidates for AICD implantation.
  • Known patients with SQTS genotypes 1-3 all had QTc intervals < 300-320 ms.
  • Known patients with SQTS genotypes 4 & 5 all had QTc intervals < 360 ms.
  • A recent review by Viskin suggested the following approach:
    • QTc intervals < 330 ms in males or < 340 ms in females should be considered diagnostic of SQTS.
    • QTc intervals < 360 ms in males or < 370 ms in females should only be considered diagnostic of SQTS when supported by symptoms or family history… But short QT syndrome is still not well understood entity.

Dr. Mandar Shah, Interventional cardiologist, Jamshedpur:

  • Short QT for sure, but to label it as Short QT Syndrome, without any family history, one has to document either VT or syncope or genetically prove it. Otherwise, ECG changes could be non-specific also.
  • Best would be to do a 7 days’ External event recorder and genetic testing before deciding any further.

CME INDIA Learning Edge

  • Short QT syndrome is a type of cardiac channelopathy that runs in families and is marked by a shortened QT interval, which can raise the chances of developing atrial and ventricular arrhythmias. Detecting this condition may pose some difficulty since healthy individuals can exhibit a wide range of QT intervals, and diagnosis usually involves analysing symptoms (like cardiac arrest and palpitations), family medical history, and a 12-lead ECG. The primary treatment option for SQTS is an implantable cardioverter-defibrillator (ICD).
  • Inherited arrhythmogenic diseases are a significant contributor to sudden death, especially among young, healthy people, and are one of the most common causes of sudden cardiac death in young athletes. These diseases include long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, and the more recently discovered short QT syndrome.
  • Diagnosing SQTS can be challenging due to the diverse clinical presentation and variability of expression among individuals and families. Cardiac arrest is the most common symptom and initial clinical presentation, followed by syncope and palpitations. Unlike long QT syndrome, there are no specific triggers for episodes that can occur at rest, during exercise, or after loud noises.
  • A key diagnostic tool for SQTS is the evaluation of the QT interval duration on a 12-lead electrocardiogram (ECG). A QT interval shorter than 330 ms in males and 340 ms in females is considered abnormal and suggestive of SQTS. However, the diagnosis cannot be made based solely on the ECG findings, and further evaluation, including genetic testing, is required.
  • In addition to genetic testing, other diagnostic tests that may be performed include exercise stress testing, Holter monitoring, and electrophysiological studies. It is also important to evaluate the patient’s family history and conduct genetic testing on family members to identify those at risk for SQTS.
  • Since SQTS can present without structural heart disease, it is crucial to consider this syndrome in patients presenting with sudden cardiac death, syncope, or palpitations, especially in young individuals. Atrial fibrillation is a common finding in SQTS and should be taken into account in the management of young patients with lone atrial fibrillation
  • Genetic testing is essential for the diagnosis of SQTS since it allows the identification of causative mutations in genes encoding for ion channels.
  • Currently, genetic testing is available for 17 genes associated with SQTS, although some of them are very rare.
  • In summary:
  • The diagnosis of SQTS is based on a combination of clinical and electrocardiographic findings, genetic testing, and the exclusion of other underlying cardiac diseases.
  • Patients with a family history of sudden cardiac death or with unexplained syncope, palpitations or cardiac arrest should be evaluated for SQTS, especially if they present with a short QT interval and/or abnormal T wave.
  • Management
    • As of now, implantable cardioverter-defibrillator (ICD) therapy is the mainstay treatment for SQTS patients.
    • However, the decision to implant an ICD in asymptomatic patients with no family history can be challenging due to the lack of clear risk stratification.
    • In such cases, a positive electrophysiological study or genetic test, along with clinical manifestations and family history, may support the decision for ICD implantation.
    • Pharmacological therapy can be considered as an alternative to ICD implantation in young children, in patients with contraindications or declining ICD implantation, as an adjunctive therapy to prevent appropriate discharges, and to prevent symptomatic episodes of atrial fibrillation. Quinidine is considered the most effective pharmacological therapy in these patients, as it blocks several potassium channels and the inward sodium and calcium currents. In SQTS 1, quinidine has been shown to produce marked prolongation of the QT interval and ventricular effective refractory periods, restoration of heart rate dependence of the QT interval, and prevention of ventricular fibrillation induction.
    • Disopyramide is another antiarrhythmic drug that has been proposed as an alternative to quinidine in SQTS 1.
    • Other antiarrhythmic drugs such as flecainide, propafenone, ibutilide, and sotalol have failed to show any beneficial effect or have shown limited efficacy

CME INDIA Tail Piece

Download Short QT Syndrome by Dr. D. P. Khaitan

References:

  1. https://www.escardio.org/Journals/E-Journal-of-Cardiology-Practice/Volume-9/Short-QT-Syndrome
  2. https://www.heighpubs.org/hjvsm/avm-aid1010.php\
  3. Gussak I, Brugada P, Brugada J, et al. Idiopathic short QT interval: a new clinical syndrome? Cardiology 2000; 94: 99–102.
  4. Redpath CJ, Green MS, Birnie DH, et al. Rapid genetic testing facilitating the diagnosis of short QT syndrome. Can J Cardiol 2009; 25: e133-e135
  5. LeoSchmroth An Introduction to Electrocardiography (Eighth adapted edition) –
    Chapter 26 Cardiac channelopathies , P 203-205
  6. Learning from studying very rare cardiac conditions: the example of short QT
    syndrome , Author : Jules C. Hancox, Dominic G. Whittaker, Henggui Zhang, Alan
    G. Stuart , Journal of Congenital Cardiology Hancox et al. Journal of Congenital Cardiology (2019)3:3
    https://jcongenitalcardiology.biomedcentral.com/track/pdf/10.1186/s40949-019-0024-
    7.pdf
  7. Short QT Syndrome An article from the E-Journal of the ESC Council for cardiology Practice
    Author – Dr. Sara Moreno Reviriego , Dr. Jose Luis Merino, FESC
    https://www.escardio.org/Journals/E-Journal-of-Cardiology-Practice/Volume-9/Short-
    QT-Syndrome
  8. Short QT Syndrome Life in the Fastlane , Ed Burns and Robert Butter , March 8, 2021
    https://litfl.com/short-qt-syndrome-ecg-library/
  9. Short QT Syndrome – Clevland Clinic
    https://my.clevelandclinic.org/health/diseases/17469-short-q-t-syndrome-sqts
  10. Short QT Syndrome – American college of Cardiology Author : Ossama K. Abou Hassan , MD; Bernard S Harbieh. Samir E. alam, MD, FACC; Marwan Re https://www.acc.org/latest-in-cardiology/articles/2016/10/05/08/06/short-qt-syndrome
  11. Short QT Syndrome – Review of diagnosis and Treatment https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4711567/
  12. Short QT Syndrome – Circulation – AHA Journals www.ahajournals.org>doi Fiorenzo Gaita, MD, Carla Giustetto, MD, Francesca Bianchi, MD Christian Wolpert, MD, Rainer Schunofm MD Elena Richiardi, and MD Martin Borggrefe MD https://www.ahajournals.org/doi/10.1161/01.cir.0000085071.28695.c4
  13. Short QT Syndrome – MedlinePlus Genetics https://medlineplus.gov/genetics/condition/short-qt-
    syndrome/#:~:text=Mutations%20in%20the%20KCNH2%2C%20KCNJ2,maintaining
    %20the%20heart’s%20normal%20rhythm.
  14. HRS/EHRA/APHRS Expert Consensus statement on the Diagnosis and Management of Patients with Inherited Primary Arrhythmia syndromes https://www.escardio.org/static-file/Escardio/Press-media/press-
    releases/2013/diagnosis-management-patients-inherited-primary-arrhythmia-
    syndromes.pdf
  15. Short QT syndrome: The current evidences of diagnosis and management Author: Ivana P. Dewi, Budi B. Dharmadjati https://onlinelibrary.wiley.com/doi/epdf/10.1002/joa3.12439


Discover CME INDIA:



Discover CME INDIA