The Value of the Electrocardiogram in Adult Congenital Heart Disease
Abstract
:1. Introduction
2. Case-Based Approach
2.1. Atrial Septal Defect (ASD)
Anatomy and Physiology
- Ostium secundum ASD is a defect in the region of the fossa ovalis. It is the most common type of ASD. Because the sinus node is in the normal position near the connection of the superior vena cava to the right atrium, it is the pacemaker of the heart and shows a sinus P wave. Additionally, because there is a normal position of the AV bundle (the bundle of His) the QRS axis is normal. An incomplete right bundle branch block (IRBBB) is 10 times more common among patients with an ASD than among healthy volunteers without cardiac disease, though there was no correlation between IRBBB and ASD diameter [1]. A notch (crochetage) in the R wave of the inferior leads (II, III and aVF) is noted seven times more often in patients with ASD than in healthy volunteers [1]. A significant correlation has been observed between ASD diameter and the presence of inferior R wave crochetage [1]. As a result of the ASD there is a left-to-right shunt that initially enlarges the right atrium and the right ventricle. This right ventricular volume overload, rather than real transmission delay, accounts for the rsR’ or rSr’ seen in patients with ASD. When the defect and the shunt are large, pulmonary hypertension can result. An ASD can permit a venous thromboembolism to become a paradoxical embolism. Cardiac auscultation of a patient with ostium secundum ASD is characterized by a systolic flow murmur in the pulmonary outflow tract and a wide-split and fixed-split S2. A wide- and fixed-split S2 means that A2–P2 (the components of S2) splitting is wide and does not vary with inspiration or with expiration. Figure 1 demonstrates a sample EKG from a patient with an ostium secundum ASD.
- b.
- Ostium primum ASD is part of the AVSD spectrum discussed below. It is the second most common type of ASD. It is a defect low in the atrial septum; however, while there is no interventricular communication, there is a cleft leaflet of the left AV valve. An ostium primum ASD occasionally has P wave changes that are consistent with left or right atrial enlargement but will often have a first-degree AV block due to prolonged intra-atrial conduction. Because of the location of the septal defect, all patients with ostium primum ASD will have an abnormal left axis. In addition, an older patient can demonstrate complete right bundle branch block [2]. Like ostium secundum ASD, cardiac auscultation of an ostium primum ASD is characterized by a systolic flow murmur in the pulmonary outflow tract and wide- and fixed-split S2. The systolic murmur of AV valve regurgitation may or may not be audible. Figure 2 demonstrates a sample EKG from a patient with an ostium primum ASD.
- c.
- Sinus venosus ASD is located in the upper atrial septum, where it connects to the superior vena cava. Of the three common types of ASD, it is the least common. It is often coupled with an anomalous right upper pulmonary vein that connects to the upper right atrium, rather than normally to the left atrium. Because of the location of the sinus venosus defect, the sinus node is absent or ectopic and the EKG rhythm is junctional or low atrial. Cardiac auscultation of a sinus venosus ASD has the same pulmonary outflow tract findings as ostium secundum and ostium primum type ASDs. These three types of ASD can be differentiated electrocardiographically.
2.2. Atrioventricular Septal Defect (AVSD)
Anatomy and Physiology
2.3. Left-Sided Valvular Heart Disease of Congenital Etiology
Anatomy and Physiology
- Bicuspid aortic valve with aortic stenosis (AS) is due to the restricted motion of this valve’s two (rather than the normal three) leaflets. During systole the aortic leaflets impede the left ventricular outflow because they cannot flatten against the ascending aorta. When the leaflet motion restriction is severe the pressure gradient across the aortic valve becomes large and the left ventricle must hypertrophy to overcome this large gradient. Auscultation of the heart of this patient demonstrates a crescendo/decrescendo systolic ejection murmur at the base of the heart and sometimes an early systolic ejection click. The EKG of a bicuspid aortic valve with severe AS shows left ventricular hypertrophy (LVH) with ST and/or T wave abnormalities secondary to hypertrophy, including ST segment depression and T wave inversion in leads I, aVL and V4–V6 (see Figure 4). When undiagnosed or untreated, the pressure-overloaded left ventricle can become dilated and dysfunctional. It is best to make the diagnosis before this left ventricular failure.
- b.
- Bicuspid aortic valve with aortic regurgitation (AR) can be due to malcoaptation of the two leaflets of this congenital aortic valve deformity, with or without associated aortic root or ascending aortic dilatation. When chronic and severe, AR renders the left ventricle volume dilated and, if left untreated, results in systolic left ventricular dysfunction. Auscultation of the heart of a patient with bicuspid aortic valve with severe AR reveals an early systolic ejection click at the lower left sternal border and a decrescendo diastolic murmur at the base of the heart, often with presystolic accentuation at the cardiac apex (Austin Flint murmur). The EKG of a bicuspid aortic valve with severe chronic AR (see Figure 5) shows LVH with tall, upright T waves in the lateral precordial leads due to left ventricular volume overload. A bicuspid aortic valve, be it with predominant AS or AR, is present in 1–2% of the population [3], but certainly a minority of patients with bicuspid aortic valve will have severe valvular stenosis or regurgitation.
- c.
- Myxomatous mitral valve disease (MMVD) or mitral valve prolapse is histologically characterized by myxomatous valve degeneration. MMVD or mitral valve prolapse is present in 2–3% of the population [4]. Most cases of MMVD are mild and asymptomatic. Myxomatous valve degeneration leads to valve redundancy which causes the leaflet(s) to prolapse, resulting in a mid-systolic click that can be followed by a mid-to-late systolic murmur at the cardiac apex. Myxomatous degeneration can be responsible for chordae tendineae rupture, resulting in a holosystolic murmur of mitral regurgitation (MR). Severe MR, when chronic, is responsible for left ventricular volume overload and, when untreated, can lead to left ventricular systolic dysfunction. The EKG of MMVD with severe chronic MR has left atrial enlargement and LVH by QRS voltage and with tall, upright T waves in the lateral precordial leads. Figure 6 is an ECG of a patient with MMVD with severe MR. Atrial fibrillation is a common presentation.
2.4. Congenital Complete Heart Block (CCHB)
Anatomy and Physiology
2.5. Tetralogy of Fallot (ToF)
Anatomy and Physiology
2.6. Congenitally Corrected Transposition of the Great Arteries (CCTGA)
Anatomy and Physiology
2.7. Hypertrophic Obstructive Cardiomyopathy (HOCM)
Anatomy and Physiology
2.8. Apical Hypertrophic Cardiomyopathy (Apical HCM)
Anatomy and Physiology
2.9. Ebstein Anomaly
Anatomy and Physiology
2.10. Pulmonary Valve Stenosis (PS)
Anatomy and Physiology
3. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
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Schiavone, W.A.; Majdalany, D.S. The Value of the Electrocardiogram in Adult Congenital Heart Disease. J. Pers. Med. 2024, 14, 367. https://doi.org/10.3390/jpm14040367
Schiavone WA, Majdalany DS. The Value of the Electrocardiogram in Adult Congenital Heart Disease. Journal of Personalized Medicine. 2024; 14(4):367. https://doi.org/10.3390/jpm14040367
Chicago/Turabian StyleSchiavone, William A., and David S. Majdalany. 2024. "The Value of the Electrocardiogram in Adult Congenital Heart Disease" Journal of Personalized Medicine 14, no. 4: 367. https://doi.org/10.3390/jpm14040367