Ventricular Septal Defect (VSD) and the ECG

Pathophysiology
Shunt flows from high pressure left ventricle to low pressure right ventricle. Shunt flow follows the left ventricle - right ventricle - pulmonary artery - left atrium - left ventricle route. ECG changes reflect the hemodynamic changes caused by the shunt flow. The ECG may be normal if VSD is small.

ECG abnormalities that may be observed in patients with VSD Right atrial abnormality (RAA). Left atrial abnormality (LAA). Right ventricular hypertrophy (RVH). Complete or incomplete right bundle branch block (RBBB). Left ventricular hypertrophy (LVH). If VSD is large, S1S2S3 pattern may be observed. Katz-Wachtel phenomenon ( sign ): large biphasic (equiphasic) QRS complexes observed in midprecordial and limb leads. Sum of the amplitudes of R and S waves in leads C2, C3 or C4 is > 60mm (6mV). Katz-Wachtel phenomenon is seen when pulmonary hypertension and the resultant biventricular hypertrophy developes.

ECG in patients with VSD and Eisenmenger syndrome Signs of right ventricular hypertrophy are observed more frequently. Signs of left ventricular hypertrophy may also accompany. The rhythm is generally sinus. Right atrial abnormality may be seen.

ECG abnormalities in adult patients who have been operated for closure of the VSD during their childhood Right bundle branch block: 50-90% if operated by right ventriculotomy 40% if operated by right atrial approach.

Complete atrioventricular (AV) block (very rare). In this group of patients, the risk of sudden cardiac death has been reported to increase in patients with left axis deviation on ECG and/or ventricular arrhythmias on Holter monitoring.

ECG 1. The ECG above, belongs to an 11 years old child who has been operated for ASD, VSD and coarctation of the aorta. Katz-Wachtel phenomenon (biventricular hypertrophy pattern) is also seen.

ECG 2. This ECG belongs to a 49 years old woman who was operated for VSD and pulmonary stenosis.

ECG 3. Twenty years old man with VSD, pulmonary stenosis and mitral stenosis. The ECG shows right bundle branch block an first degree AV block.

ECG 4. The above ECG belongs to an 8 years old boy. He was operated for Transposition of the Great Arteries and Ventricular Septal Defect (VSD) when he was 1 year old. VSD closure operations increase the risk of right bundle branch block.

Echocardiography
Two-dimensional (2D) and Doppler color-flow mapping may be used to identify the type of defect in the ventricular septum. Perimembranous VSDs are characterized by septal dropout in the area adjacent to the septal leaflet of the tricuspid valve and below the right border of the aortic annulus.

The subaortic or anterior malalignment type of VSD appears just below the posterior semilunar valve cusps, entirely superior to the tricuspid valve. Subpulmonary VSD appears as echo dropout within the outflow septum and extending to the pulmonary annulus. One or two of the aortic cusps may be seen to be protruding through the defect into the right ventricular outflow tract. The inlet AV septal-type of VSD extends from the fibrous annulus of the tricuspid valve into the muscular septum; it is often entirely beneath the septal tricuspid leaflet. Muscular defects may appear anywhere throughout the ventricular septum. They may be either large and single or small and multiple. The anatomic localization of all VSDs is facilitated by coupling 2D sonograms with a Doppler system and by superimposing a color-coded direction and velocity of blood flow on the real-time images.

Chest radiography
In patients with small VSDs, the results of chest radiographs are usually normal. With medium-size VSDs, minimal cardiomegaly and a borderline increase in pulmonary vasculature may be observed. In large VSDs, the chest radiograph shows gross cardiomegaly with prominence of both ventricles, the left atrium, and the pulmonary artery. The pulmonary vascular markings are increased, and frank pulmonary edema, including pleural effusions, may be present.

Electrocardiography
The electrocardiogram (ECG) mirrors the size of the shunt and the degree of pulmonary hypertension. Small restrictive VSDs usually produce a normal tracing. Medium-sized VSDs produce a broad, notched P wave characteristic of left atrial overload. Signs of LV volume overload namely, deep Q and tall R waves with tall T waves in leads V5 and V6 are present. In addition, signs of atrial fibrillation are often present. Large VSDs produce right ventricular hypertrophy with right-axis deviation. With further progression, the ECG shows biventricular hypertrophy; P waves may be notched or peaked.

Tetralogy of Fallot (ToF) and the ECG

ECG abnormalities that are frequently observed in patients with ToF

Atrial arrhythmias are frequently observed after corrective surgery. Ventricular arrhythmias may be observed after corrective surgery. If QRS width is > 180 msec, this suggests an increased risk for development of ventricular arryhthmias and sudden cardiac death.

ECG 1. The ECG of a 7 years old boy who was operated for ToF one year ago. He is under congestive heart failure therapy

Diagnosis
Hemoglobin and hematocrit values are usually elevated in proportion to the degree of cyanosis. Patients with significant cyanosis have the following, in association with a tendency to bleed: Decreased clotting factors Low platelet count Diminished coagulation factors Diminished total fibrinogen Prolonged prothrombin and coagulation times Arterial blood gas (ABG) results are as follows: Oxygen saturation varies pH and partial pressure of carbon dioxide (pCO2) are normal unless the patient is in extremis Imaging studies include the following: Echocardiography Chest radiographs Magnetic resonance imaging (MRI) Echocardiography has the following attributes: Color-flow Doppler echocardiography accurately diagnoses ductus arteriosus, muscular VSD, or atrial septal defect

The coronary anatomy can be revealed with some degree of accuracy Valvar alterations can be detected with ease In many institutions, echocardiography is the only diagnostic study used before surgery Chest radiographs have the following attributes:

Often normal initially Diminished vascularity in the lungs and diminished prominence of the pulmonary arteries gradually become apparent The classic boot-shaped heart (coeur en sabot) is the hallmark of the disorder MRI has the following attributes: Provides good delineation of the aorta, RVOT, VSDs, RV hypertrophy, and the pulmonary artery and its branches[3] Can also be used to measure intracardiac pressures, gradients, and blood flows Cardiac catheterization is extremely useful in any of the following instances: The anatomy cannot be completely defined by echocardiography Disease in the pulmonary arteries is a concern Pulmonary vascular hypertension is possible Cardiac catheterization findings include the following: Assessment of the pulmonary annulus size and pulmonary arteries Assessment of the severity of RVOTO Location of the position and size of the VSD Ruling out possible coronary artery anomalies
SPELL

Background

The Tet spell (also called hypoxic spell, cyanotic spell, hypercyanotic spell or paroxysmal dyspnea) most frequently occurs in young infants with Tetralogy of Fallot but may occur with other congenital heart defects that have pulmonary or subpulmonary stenosis and a VSD, and at any age. Common precipitants include crying, defecation, feeding, waking from naps (low systemic resistance), fever, dehydration, tachypnoea / tachycardia due to any cause, and medications (e.g. ACE inhibitors). They tend to occur in those with mild-to-moderate cyanosis at rest and are more common in children who are iron deficient. They are characterised by: Period of uncontrollable crying / panic, Rapid and deep breathing (hyperpnoea), Deepening of cyanosis, Decreased intensity of heart murmur, Limpness, convulsions and rarely, death. Hypercyanotic spells need to be recognised quickly and effectively controlled to prevent the development of serious complications from prolonged hypoxia. While medical intervention is indicated, many episodes are self-limiting. Those involved in the care of a child with Tetralogy of Fallot should be familiar with a spells presentation and early management.

Pathophysiology

Hypercyanotic spells may be best thought of as an imbalance between pulmonary and systemic vascular resistance favouring decreased pulmonary flow and increased right-to-left shunting. Hypoxemia, metabolic acidosis, hyperpnoea, increased systemic venous return, catecholamines, and pulmonary vasoconstriction are thought to be involved in an interaction that results in a self-perpetuating cycle. Infundibular spasm is not required.

TUJUAN TERAPI DIET Memberikan makanan secara bertahap (energi & konsistensi) tanpa memberatkan kerja jantung Mencegah/mengurangi penimbunan cairan dengan membatasi asupan cairan dan garam natrium Mengurangi kembung dengan menghindari makanan yang mengandung gas PRINSIP/SYARAT DIET . Energi cukup 2. Protein cukup : 1 g/kgBB/hari , bila ada insuf renal protein 0,8 /kgBB/hari 3. Cairan: dibatasi, bila ada edema paru keseimbangan cairan harus negatif, cairan yang masuk (infus & minuman/makanan) lebih sedikit dari pada cairan yang keluar (Urine & IWL) 4. Vitamin, mineral & elektrolit a. Natrium dibatasi: 1500 2000 mg/hari b. c. Kalium: 2000 6000 mg/hari Magnesium: 300 350 mg/hari

5. Bentuk makanan diberikan secara bertahap sesuai kemampuan dapat dimulai dari lunak bubur, tim dan nasi
PENATALAKSANAAN MEDIK a. Terapi Non-Farmakologis Anjuran umum 1. Edukasi : terangkan hubungan keluhan, gejala dengan pengobatan 2. Aktivitas sosial dan pekerjaan diusahakan agar dapat dilakukan seperti biasa sesuai kemampuan fisik 3. Gagal jantung berat harus menghindari penerbangan panjang Tindakan umum 1. Diet rendah garam 2. Hentikan rokok 3. Aktivitas fisik 4. Istirahat baring pada gagal jantung akut, berat, dan eksaserbasi akut b. Terapi Farmakologis 1. Glikosida jantung Digitalis, meningkatkan kekuatan kontraksi otot jantung dan memperlambat frekuensi jantung Efek yang dihasilkan : peningkatan curah jantung, penurunan tekanan vena dan volume darah, dan peningkatan diurisi dan mengurangi edema. Ex : Digoxin - Digoxin memiliki efek inotropik positif (bekerja meningkatkan kontraksi otot jantung ) pada irama sinus dan menyebabkan perbaikan simptomatis serta menurunkan tingkat perwatan di rumah sakit walaupun tidak mempengaruhi tingkat mortilitas. - Penghambat Fosfodiesterase Hambatan enzim ini menyebabkan peningkatan kadar siklik AMP (cAMP) dalam sel miokard yang akan meningkatan kadar kalsium intrasel, ex : Mirinon dan Amirinon 2. Diuretik

Dasar untuk terapi simptomatik. Dosisnya harus cukup besar untuk menghilangkan edema paru dan/atau perifer. Efek samping utama adalah hipokalemia ( berikan suplemen K+ atau diuretik hemat kalium seperti amilorid) Ex : Spironolakton, suatu diuretik hemat kalium (antagonis aldosteron), memperbaiki prognosis pada CHF berat. 3. Inhibitor ACE Menghambat perubahan angiotensin I menjadi angiotensin II, memotong respon neuroendokrin maladaptif, menimbulkan vasodilatasi dan menurunkan tekanan darah. Obat ini dapat memicu gagal ginjal pada stenosis arteri renalis bilateral. Efek samping lain : batuk kering persisten 4. Antagonis Reseptor Angiotensin II Ex : Losartan, menghambat angiotensin II dengan antagonisme langsung terhadap reseptornya. Efek dan manfaatnya sama seperti inhibitor ACE. 5. -Bloker Ex : Bisoprolol, Metoprolol, Karvedilol -Bloker diberikan hanya pada pasien yang stabil, dengan dosis sangat rendah, dinaikkan bertahap. Menurunkan kegagalan pompa serta kematian mendadak akibat aritmia. 6. Kombinasi Hidralazin dengan Isosorbid Dinitrat Untuk pasien yang intoleran dengan inhibitor ACE 7. Terapi Umum Obati penyebab yang mendasari dan aritmia bila ada. Kurangi asupan garam dan air, pantau terapi dengan mengukur berat badan setiap hari. Obati faktor resiko hipertensi dan PJK dengan tepat.