This ECG was recorded from a 5 week old baby boy, the birth of the child was a normal delivery with no complications, baby was born full term. From a few weeks old, he started being irritable during breast feeding and Mum noticed that the baby became diaphoretic during feeds and also seemed to be in a lot of distress, but the baby's mother just thought this was due to the baby having "reflux"
Baby attended the Emergency Department due to having a low grade fever, rhinorrhea and poor feeding. On examination, he was found to have increased respiratory effect and he was tachycardic, also a soft systolic murmur was found and a few crepitations in the patient's lungs. Liver is palpable 3cm below the costal margin.
What's your diagnosis?
Baby attended the Emergency Department due to having a low grade fever, rhinorrhea and poor feeding. On examination, he was found to have increased respiratory effect and he was tachycardic, also a soft systolic murmur was found and a few crepitations in the patient's lungs. Liver is palpable 3cm below the costal margin.
What's your diagnosis?
There seems to be significant hypertrophy and/or dilation noted. Left atrial enlargement evident by biphasic p wave in V1. Right atrial enlargement suspected by assessment of p wave in inferior leads. Probable LVH. Possible RVH, there is a large R wave in V1 and poor R wave progression noted. There is ST elevation in V4 and V5. The history is also suggestive of an output anomaly. Structural?
ReplyDeleteThere are large Q waves in V4 to V6, I and aVL and ST elevation in V4 to V6 - Classical ECG of ALCAPA (anomalous left coronary artery arising from pulmonary artery. Also, a QT pattern (Q waves deeper than 3 mm with an inverted T wave) in avL is specific to this entity and differentiates from dilated cardiomyopathy. The child needs to undergo an urgent echocardiography followed by urgent surgical repair of the anomaly.
ReplyDeleteDr Kartikeya Bhargava
Consultant Cardiac Electrophysiologist, Delhi INDIA
classical ecg for alcapa. agree with kartikeya. will like to add- chang and allada proposed a scoring system to differentiate from nonischemic cmp. q> 30ms in lead I and deeper than 3 mm in aVL were common with alcapa. needs urgent echo for confirming diagnosis and urgent surgery
ReplyDeleteit is normal ECG in which sinus tachycardia in baby...till it is proven by echo...
ReplyDeleteThis is NOT a normal ECG, (instead, the sinus tachycardia is a normal finding for this age).
ReplyDeleteI agree with the most likely dx. from this ECG alone being anomalous origin of LCA arising from PA.
I was wondering if this patient might have a VSD. An echo would clearly be a start of the diagnostic modality. A left to right shunt might account for the left sided hypertrophy when in fact this infant should have RVH and R:S ration greater then 1 in V1.
ReplyDeleteLVH/LV dilatation as per voltage criteria, ALCAPA may be possible, as baby has soft systolic murmur congenital aortic stenosis or HOCM is also predictable. ALCAPA with VSD is also possible or only VSD is also. So echo is the only choice to outrule.
ReplyDeleteVinay.
Cardiac Physiologist
Voltage criteria high on all leads, is this due to small body size and short displacement between individual electrodes - electrodes < 15 cm apart on limb leads, distortion of Wilson's central terminal for pre cordial leads.
ReplyDeleteNegative p wave in V1 is aberrrant, QRS width normal, could be AVRT concealed pathway ? close to septum. If I remember correctly normal HR for patient this age should be approx 100 bpm. Neo nates have far lower blood volumes than adults - therefore rate is relatively tachycardic ? enough to reduce LV filling volume and subsequent stroke volume - ? plausible cause of symptoms.
ST elevation V4 - V6, allowing for neo natal right axis deviation, would suggest LAD MI but how connected to AVRT? ST elevation associated with Brugada as well as IHD (in adult V1), could also be high ST take off and therefore incidental to situation.
Would do echo to look for any structural defects, aniogram to check for coronary perfusion, then pursue with EP mapping of tachycardia.
Creps, SSM, bi-atrial enlargment, LVH with strain pattern; LV dilatation. Likely large shunt. ? AV valve regurgitation. Doubt it is outflow obstruction. Clinical background and low diagnostic ECG certainly warrants urgent echo. One would think the variation in interpretation between us is ominous, I find it reassuring that we all agree on further sonographic imaging! Hope the poor bugger doesn't Eisenmengers.
ReplyDeleteThe child needs to be evaluated for Tracheoesophageal Anomalies, for the following reasons..
ReplyDelete1. Rhinorrhea / accumulations of secretions;
2. Sporadic or continuous respiratory distress during and following feedings with regurgitation
3. Fever may indicate infection due to aspiration
Tachypnea, Tachycardia, Heptomegaly, Poor/ slow feeding, poor weight gain are possible Acyanotic Heart Disease.
ReplyDeleteif with such symptoms and such ecg alcapa is not suspected then u have missed the bus and so has the baby
ReplyDeleteMany thanks for all your comments... Answer to this case study:
ReplyDeleteThe clinical scenario paint's a picture of cardiac failure. ECG has a anterolateral infarct pattern, with deep Q wave in I, aVL and Lead 3,4,5,6. This ECG pattern in newborn is very suggestive of ALCAPA. Baby is a 5 weeks old baby. He was born at term with normal delivery with no complication's. From 3 weeks old, he started to be irritable especially during breast feeding. Mum noticed that he was sweating during feeds, so ALCAPA was the most likely possible diagnosis. Irritability and sweating during feeding is a sign of heart failure, so we should also think of ischemia on the ECG and the anterolateral infarction, this is a rare diagnosis in someone so young.
Dx: Anomalous Left Coronary Artery arising from Pulmonary Artery (ALCAPA), CHF, Anterolateral infarction.
Some key learning points about (ALCAPA):
▪ The anomaly is also called Bland-White-Garland syndrome and is a rare but serious congenital anomaly, accounting for approximately 0.25-0.5% of all congenital heart disease..
▪ ALCAPA is dramatically improved as a result of both early diagnosis using echocardiography with color flow mapping and improvements in surgical techniques, including myocardial preservation.
▪ ALCAPA is usually an isolated cardiac anomaly but, in rare incidences, has been described with Patent ductus arteriosus, Tetralogy of Fallot, Coarctation of the aorta and Ventricular Septal Defect (VSD).
▪ The left ventricular myocardium is being perfused by relatively desaturated blood under low pressure, leading to myocardial ischemia, during periods of increased myocardial demands, such as when the infant is feeding and crying. Further increases in myocardial oxygen consumption can lead to infarction of the anterolateral left ventricular free wall. This often causes mitral valve papillary muscle dysfunction and variable degrees of mitral insufficiency. As a result, left ventricular myocardium remains underperfused. Consequently, the combination of left ventricular dysfunction and significant mitral valve insufficiency leads to congestive heart failure (CHF).
▪ ALCAPA is not considered an inheritable congenital cardiac defect. No risk factors for the occurrence of ALCAPA in any individual family are known, and ALCAPA is not associated with any syndromes or noncardiac conditions.
▪ Left untreated, the mortality rate in the first year of life is 90% secondary to myocardial ischemia or infarction and mitral valve insufficiency leading to CHF. Sudden death may occur because of inadequate collateral circulation between the left and right coronary artery systems.
▪ Tachypnea, poor feeding, irritability, diaphoresis) in the young infant. Inadequate myocardial perfusion likely causes significant chest pain and these symptoms of myocardial ischemia may be misinterpreted as routine infantile colic.
▪ Children outgrow these symptoms and gradually become asymptomatic, although periodic dyspnea, angina pectoris, syncope, or sudden death may still occur in adulthood.
▪ Approximately 85% of patients present with clinical symptoms of CHF within the first 1-2 months of life. In unusual cases, the clinical presentation with symptoms of myocardial ischemia may be delayed into early childhood. Rarely, a patient may stabilize following infarction and present with mitral valve regurgitation later in childhood or even adulthood.
Biphasic p wave in V1 is evident by left atrial enlargement.Right atrial enlargement suspected by assessment of p wave in inferior leads. LVH or dilation LV. Possible RVH for T wave positive and large R wave in V1 and poor R wave progression. There is ST elevation in V4 and V5.
ReplyDeleteTachypnea, Tachycardia, Hepatomegaly, Poor/ slow feeding, systolic ... Mostra tuttomurmur and a few crepitations in the lungs are typical for Acyanotic Heart Disease with Cardiac Failure.
CHF in isolated ALCAPA (Anomalous Left Coronary Artery arising from Pulmonary Artery) is unusual in 5 weeck, but it's possible if associated a coartaction of the aorta and PDA (Patent ductus arteriosus).
ALCPA
ReplyDelete