Journal of Electrocardiology

Electrocardiogram in Patients with Pulmonary Hypertension

--Manuscript Draft--

Manuscript Number: JECG-D-23-00015

Article Type: Review Article

Keywords: pulmonary hypertension; PH; electrocardiogram; ECG

Abstract: Introduction
Pulmonary hypertension (PH) is a potentially life-threatening cardiovascular disease
defined by a mean pulmonary arterial pressure (mPAP) > 20 mmHg. Due to
nonspecific symptoms, PH is often diagnosed late and at advanced stage. In addition
to other diagnostic modalities, the electrocardiogram (ECG) can help in establishing
the diagnosis. Knowledge of typical ECG signs could help to detect PH earlier.
Methods
A selective literature review on the typical electrocardiographic patterns of PH was
performed.
Results
Characteristic signs of PH include right axis deviation, SIQIII and SISIISIII patterns, P
pulmonale, right bundle branch block, deep R waves in V1 and V2, deep S waves in
V5 and V6, and right ventricular hypertrophy (R in V1 + S in V5, V6 > 1,05 mV).
Repolarisation abnormalities such as ST segment depressions or T wave inversions in
leads II, III, aVF, and V1 to V3 are common as well. Furthermore, a prolonged QT/QTc
interval, an increased heart rate, or supraventricular tachyarrhythmias can be
observed. Some parameters may even provide information about the patient's
prognosis.
Conclusion
Not every PH patient shows electrocardiographic PH signs, especially in mild PH.
Thus, the ECG is not useful to completely rule out PH, but provides important clues to
PH when symptoms are present. The combination of typical ECG signs and the co-
occurrence of electrocardiographic signs with clinical symptoms and elevated BNP
levels are particularly suspicious. Diagnosing PH earlier could prevent further right
heart strain and improve patient prognosis.

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Title Page
Title: Electrocardiogram in Patients with Pulmonary Hypertension

Authors
Lukas Ley1
1.) Justus-Liebig-Universität Gießen, Campus Kerckhoff, Benekestrasse 2-8, 61231 Bad
Nauheim, Germany

Reinhard Höltgen2
2.) Klinikum Westmünsterland, St. Agnes-Hospital Bocholt Rhede, Medical Clinic,
Cardiology/Electrophysiology, Barloer Weg 125, 46397 Bocholt, Germany

Harilaos Bogossian3
3.) School of Medicine, Cardiology Department, Witten/Herdecke University, Witten,
Germany

Hossein Ardeschir Ghofrani4

4.) Medizinische Klinik und Poliklinik II, Universitätsklinikum Giessen, Klinikstraße 33,
35392, Giessen, Deutschland

Dirk Bandorski5,6*
5.) Faculty of Medicine, Semmelweis University Campus Hamburg, Lohmühlenstraße 5/Haus
P, 20099 Hamburg, Germany
6.) Neurological Clinic Bad Salzhausen, Am Hasensprung 6, 63667 Nidda, Germany

*
= corresponding author

Correspondence to:
Dr. med. Dr. habil. Dirk Bandorski, FACC, FESC, MHBA
Faculty of Medicine, Semmelweis University Campus Hamburg, Lohmühlenstraße 5/Haus P,
20099 Hamburg, Germany
Phone: +49 6043-804212, Fax: +49 6043-804236
e-mail: d.bandorski@asklepios.com
keywords: pulmonary hypertension, PH, electrocardiogram, ECG
Abstract
Introduction
Pulmonary hypertension (PH) is a potentially life-threatening cardiovascular disease defined
by a mean pulmonary arterial pressure (mPAP) > 20 mmHg. Due to nonspecific symptoms,
PH is often diagnosed late and at advanced stage. In addition to other diagnostic modalities,
the electrocardiogram (ECG) can help in establishing the diagnosis. Knowledge of typical
ECG signs could help to detect PH earlier.
Methods
A selective literature review on the typical electrocardiographic patterns of PH was
performed.
Results
Characteristic signs of PH include right axis deviation, SIQIII and SISIISIII patterns, P
pulmonale, right bundle branch block, deep R waves in V1 and V2, deep S waves in V5 and
V6, and right ventricular hypertrophy (R in V1 + S in V5, V6 > 1,05 mV). Repolarisation
abnormalities such as ST segment depressions or T wave inversions in leads II, III, aVF, and
V1 to V3 are common as well. Furthermore, a prolonged QT/QTc interval, an increased heart
rate, or supraventricular tachyarrhythmias can be observed. Some parameters may even
provide information about the patient's prognosis.
Conclusion
Not every PH patient shows electrocardiographic PH signs, especially in mild PH. Thus, the
ECG is not useful to completely rule out PH, but provides important clues to PH when
symptoms are present. The combination of typical ECG signs and the co-occurrence of
electrocardiographic signs with clinical symptoms and elevated BNP levels are particularly
suspicious. Diagnosing PH earlier could prevent further right heart strain and improve patient
prognosis.
Introduction
Pulmonary hypertension (PH) is a potentially life-threatening cardiovascular disease. A mean
pulmonary arterial pressure > 20 mm Hg, measured by right heart catheterisation, is required
for diagnosis. PH is classified into five groups based on the underlying pathomechanism. The
characteristic symptom of PH is exertional dyspnoea; however, numerous other heart failure
symptoms are possible. Because of the non-specific and often mild symptoms, PH is commonly
diagnosed late, delaying adequate therapy and exposing the right heart to further strain [1].
Since the functional state of the right heart is a major determinant of the patient's prognosis, the
earliest possible therapy should be a physician‘s aim [2, 3]. Due to its ubiquitous availability,
easy and inexpensive performance, the simple electrocardiogram can contribute to the diagnosis
of PH and thus ideally improve the prognosis of PH patients. Furthermore, studies have shown
that the ECG can also estimate hemodynamic severity as well as predicting prognosis and
indicate a response to therapy [1, 4-6]. In the following, typical electrocardiographic findings
of chronic right heart strain in PH are presented.
Specialties in displaying the right heart in an ECG
In a healthy heart, the left ventricle dominates the ECG due to its larger muscle mass; the thick-
walled left ventricle masks the thin-walled right ventricle. But, as soon as the right ventricle is
exposed to increased volume and/or pressure, as it is in PH, it must first hypertrophy to two to
threefold to accumulate a larger muscle mass than the left ventricle. Under these conditions, the
right ventricle pulls the electrical forces more forward and is able to induce visible
electrocardiographic changes [7-9]. This usually takes severe right ventricular strain, i.e. an
advanced stage of PH [7]. In a heart exposed to right heart strain, lead V1 usually shows
electrocardiographic pathology most clearly and earliest, as it is the lead closest to the right
heart [7, 9]. Extending the ECG to leads V3R-V4R may be diagnostically helpful [7, 10].
Note: Electrocardiographic signs of PH appear only in advanced disease and are best seen in
leads V1 and V3R-V4R.
Rhythm
Most patients suffering from PH are in sinus rhythm at the time of diagnosis [11, 12]. However,
arrhythmias have been reported to occur in PH patients, especially as the disease progresses
[13]. Most commonly observed are supraventricular tachyarrhythmias such as atrial fibrillation
and atrial flutter [14]. Less commonly, ventricular arrhythmias are found [15]. Transient
supraventricular arrhythmias can lead to clinical deterioration, which can affect quality of life
dramatically. If the arrhythmias remain long-term, they impair patient prognosis. Therefore,
patients benefit from rhythm-controlling therapy in both cases [14]. Sudden cardiac death
(SCD) is a common cause of death in PH patients, but is usually independent of previous
arrhythmias [16].
Note: Arrhythmias are not a specific sign of PH, but indicate advanced PH and should be treated
with rhythm-controlling therapy to improve quality of life and prognosis.
Heart rate
Average heart rate may be elevated in PH patients [13]. Increased heart rate or tachycardia is
associated with a worse prognosis in PH [11, 17]. However, drug treatment to lower the heart
rate in PH is currently not recommended [1].
Note: An increased heart rate is not a specific sign of PH, but may indicate a worse prognosis
in PH patients.

Axis deviation
Axis deviations associated with right heart strain are characteristic features of PH. These
include right axis deviation (> 90°), and the SIQIII or SISIISIII pattern [7, 9]. Since right axis
deviation can also occur in young or thin adults, children, infants, COPD patients, patients with
lateral myocardial infarction and patients with LPHB, and a SIQIII pattern can also occur in
acute pulmonary embolism, an axis deviation should be evaluated with additional
electrocardiographic signs [9].
Note: A right axis deviation, of the SIQIII/SISIISIII pattern are typical features of PH, but
should be evaluated with further electrocardiographic information to rule out differential
diagnoses.

Right axis deviation (paper speed: 50 mm/s; 10 mm = 1 mV)

SIQIII pattern (paper speed: 50 mm/s; 10 mm = 1 mV)

SISIISIII pattern (paper speed: 50 mm/s; 10 mm = 1 mV)

Atrial signs
Another typical electrocardiographic indicator of PH is an increase in P-wave amplitude. If the
P wave exceeds an amplitude of 0.25 mV in the limb leads (especially lead II) or an amplitude
of 0.15 mV in the precordial leads (especially V1) P pulmonale, also called P dextroatriale, is
present [1, 9]. A P pulmonale predicts a worse prognosis in PH [18]. The P wave duration
usually remains normal [19]. However, if there is an additional cardiovascular disease affecting
the left atrium, a so-called P biatriale may also occur. A P biatriale is characterised by a
prolonged P wave duration (> 120 ms) in addition to an increased P wave amplitude [9].
Note: P pulmonale (> 0.25 mV in lead II or > 0.15 mV in lead V1) is a characteristic feature of
PH. In case of combined right and left atrial strain, a P biatriale may also occur (increased P
wave amplitude and prolonged P wave duration > 120 ms).

P pulmonale (paper speed: 50 mm/s; 10 mm = 1 mV)

Ventricular signs
QRS time may be prolonged in PH patients and can present as incomplete or complete right
bundle branch block, indicating PH [1, 9]. Prolonged QRS time in PH is associated with higher
mortality [20, 21]. Other typical ECG changes include tall R waves in leads V1 and V2 and
deep persistent S waves in leads V5 and V6 [22]. From these characteristics, the modified
Sokolow-Lyon criteria for right ventricular hypertrophy (R V1, V2 + S V5,V6) is formed [23].
Moreover a qR configuration in lead V1 is frequently seen in PH and is associated to a worse
prognosis [24].
Note: (Incomplete) right bundle branch block provides evidence of PH and is associated with a
worse prognosis. Moreover tall R waves in leads V1 and V2 and deep S waves in leads V5 and
V6 as well as a qR configuration in lead V1 are typical findings in PH.

qR in V1 (paper speed: 50 mm/s; 10 mm = 1 mV)

Repolarisation signs
Both ST segment depressions and T wave inversions can be observed in PH [1, 9]. Both together
are referred to as a right ventricular strain pattern [1]. T wave inversions in the inferior (II, III,
aVF) and anterior leads (V1-3) are typical [1]. However, extension to the lateral precordial leads
may also be observed [25]. The more leads affected by the T wave inversions, the more
specifically this may indicate PH and suggest a worse prognosis [25, 26]. These T wave
inversions are also referred to as “snow shovel-like“ T waves due to their characteristic shape.
The main differential diagnosis of such nonspecific repolarisation abnormalities is chronic
coronary syndrome (CCS), although these patterns of repolarisation abnormalities in CCS are
rarely associated with other right ventricular strain signs [27].
Note: ST segment depressions and T wave inversions are frequently seen in PH. The more leads
showing T wave inversions, the more specific for PH and the worse the prognosis. However, it
is important to exclude chronic coronary syndrome as a cause of these changes.

“Snow shovel-like“ T wave inversions in V1-V5 (paper speed: 50 mm/s; 10 mm = 1 mV)

Intervals
The PR interval is not altered in any particular way in PH patients, on average [18]. However,
QT or QTc interval may be prolonged in PH [1, 28]. Prolongation of the QTc interval may be
associated with ventricular arrhythmias and SCD [29]. Prolongation of QT or QTc interval
suggests a worse prognosis in PH [28, 30].
Note: Prolongation of the QT/QTc interval is not specific for PH, but suggests a worse
prognosis.

Right axis deviation, P pulmonale, right bundle branch block, QT interval: 520 ms, T wave
inversions in V1-V5 (paper speed: 50 mm/s; 10 mm = 1 mV)
More complex ECG criteria of right heart strain
If typical criteria indicating PH occur, this can be confirmed by the analysis of more complex
ECG parameters. Such parameters indicate right ventricular or right atrial hypertrophy or strain
and are shown in the table below.
ECG criteria Cut-off Source
R aVR > 0.4 mV Sokolow [23]
R V1 > 0.6 mV Myers [31]
R V5,V6 < 0.3 mV Myers [31]
S V1 < 0.2 mV Myers [31]
S V5 > 1.0 mV Myers [31]
S V6 > 0.3 mV Myers [31]
R/S V1 > 1.0 Myers [31]
R/S V5 < 0.75 Myers [31]
R/S V6 < 0.4 Myers [31]
R/S V5 : R/S V1 < 0.04 Sokolow [23]
(R I + S III) - (S I + R III) < 1.5 mV Lewis [32]
Max R V1,2 + max S I,aVL - S V1 > 0.6 mV Butler [33]
R V1 + S V5,6 > 1.05 mV Sokolow [23]
R peak V1 (QRS < 120ms) > 35 ms Myers [31]
RSR‘ V1 (QRS > 120ms) Present Hancock [19]
S > R in I, II, III Present Hancock [19]
S I and Q III Present Hancock [19]

Conclusion
Because the characteristic electrocardiographic changes of right heart strain do not occur until
advanced stages of disease, not every PH patient shows these electrocardiographic signs,
especially in mild PH [34, 35]. However, in more severe PH, typical ECG signs are observed
at a high frequency. One study reported that, shortly before death, the ECG was abnormal in
every PH patient [13]. Thus, the ECG is not useful to completely rule out PH, but provides
important clues to PH when symptoms are present [1, 36, 37]. The combination of typical ECG
signs and the co-occurrence of electrocardiographic signs with clinical symptoms (i.e.
exertional dyspnoea) and laboratory results (i.e. elevated BNP levels) are particularly suspect,
because without this additional information, the above ECG criteria have high specificity but
low sensitivity [7, 9, 19, 38, 39].
Note: ECG is not able to completely exclude the PH diagnosis, but may suggest PH in advanced
disease, especially in combination with typical symptoms and elevated BNP.
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