Patent ductus arteriosus differential diagnosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief:Cafer Zorkun, M.D., Ph.D. [2]; Keri Shafer, M.D. [3] Priyamvada Singh, MBBS[4] Ramyar Ghandriz MD[5]

Overview

Patent ductus arteriosus is very famouse due to its continuous machine-like murmur. There are some certain conditions that may make continuous murmur.

Differentiating Patent Ductus Arteriosus from other Diseases

• Patent ductus arteriosus should be differentiated from other conditions producing machine-like continuous murmur.

Differentiating Patent ductus arteriosus from other acyanotic congenital heart diseases:

Diseases	Clinical manifestations						Para-clinical findings			Gold standard	Additional findings
	Symptoms			Physical examination
							Imaging
	Exertional dyspnea	Failure to thrive	Recurrent respiratory infections	Murmur on auscultation	Peripheral edema	Clubbing	Echocardiography	Chest x-ray	Cardiac CT
Patent ductus arteriosus	Not at beginning May be produced during the course of disease	Depends on the size	-	Continuous machine-like murmur	-	May be present by progressing	In color-Doppler visualization of flow through the patent duct which has a high velocity	Non-specific	Used for determining Krichenko classification based on PDA size and anatomical features.	Echocardiogram	Krichenko criteria for classification is a very important factor for treatment
Atrial septal defect	+/−	+/−	+/−	Systolic flow murmur in the upper left sternal border Wide, fixed splitting of S2 Diastolic flow rumble across the tricuspid valve	+/−	+/−	Hypermobile interatrial septum Abrupt septal irregularity Right atrial and ventricular volume overload Pulmonary artery dilatation	Cardiomegaly Pulmonary artery enlargement/increased pulmonary vascularity	Enlargement of the right atrium and ventricle	Echocardiogram	Atrial septal defect is classified into 5 types including ostium primum defect, ostium secundum defect, superior sinus venosus defect, inferior sinus venosus defect, and coronary sinus defect
Ventricular septal defect	-/+	-/+	After Eisenmenger syndrome	Holosystolic murmur May mimic aortic stenosis(mid/end dyastolic murmur due to increased pulmonary circulation)	-/+	-/+	Defect localization septal dropout in the area adjacent to the tricuspid septal leaflet and below the right border of the aortic annulus Direction of jet: Left to right / right to left	Cardiomegaly in large VSD	Direct visualisation of murmur	Echocardiogram	-
Diseases	Exertional dyspnea	Failure to thrive	Recurrent respiratory infections	Murmur on auscultation	Peripheral edema	Clubbing	Echocardiography	Chest x-ray	Cardiac CT	Gold standard	Additional findings
Coarctation of the aorta	+/−	+/−	−	Systolic murmur over the upper sternal border with radiation to the back Murmur of mitral regurgitation (holosystolic murmur best heard at the apex)	-	-	Narrowing of the aortic arch at the level of the isthmus Left ventricular hypertrophy	Notching of the posterior fourth to eighth ribs due to dilated intercostal arteries Indentation of the aorta at the site of coarctation with pre- and post-stenotic dilation of the aorta (classic "3 sign")	Dilation of the intercostal arteries	Echocardiogram	Patients present with arm-leg blood pressure gradient of >20mmHg
Patent foramen ovale	−	−	−	−	−	−	Appearance of at least 3 micro-bubbles in the left atrium within three cardiac cycles after the complete opacification of the right atrium	Non specific	−	Echocardiogram	It is associated with migraine headache and decompression sickness in divers
Aortic stenosis	+	+	+	Crescendo-decrescendo mid-systolic (or ejection systolic) murmur	+/-	Depending on severity	Used for finding the location of stenosis Finding severity Evaluating the flow jet with color-Doppler ultrasound technique	Non-specific at the beginning At progressed stage calcification of the valve and cardiomegally	Calcification score	MRI	MRI provides a more detailed structural and dynamic assessment of the aortic valve and left ventricle, in particular
Pulmonary stenosis	Depending on severity	-	-/+	Continuous systolic murmur	-	-/+	Right atrial hypertrophy	Non-specific	Direct visualization of stenosis	Echocardiogram	-

Differential diagnosis of patent ductus arteriosus from other continuous machine-like murmur:

Diseases	Clinical manifestations						Para-clinical findings			Gold standard	Additional findings
	Symptoms			Physical examination
							Imaging
	Exercise intolerance	Respiratory distress	Failure to thrive	Cardiac auscultation	Cyanosis	Wide pulse pressure	Chest X-ray	Doppler Echocardiography	CT-scan
Patent Ductus Arteriosus	In medium to large size	In large size	In large size	Continuous Machine-like murmur	+	+	Left sided cardiomegaly	Left to right shunt	krichenco criteria	Charachtristic murmur	Increased QT-intrerval maybe seen
Venus Hum	-	-	-	Continuous murmur	-	-	Normal	Normal	Normal
Mammary Souffle	-	-	Does not apply	Continuous murmur	-	-/+	Normal	Normal	Normal		Pregnancy related
Diseases	Exercise intolerance	Respiratory distress	Failure to thrive	Machine-like murmur	Cyanosis	Wide pulse pressure	Chest X-ray	Doppler Echocardiography	CT-scan	Gold standard	Additional findings
Aortopulmonary Window	+	+	+	Continuous murmur	+	+	Cardiomegaly	Left to right shunt, Eisenmenger's syndrome	Aortic aneurysm
Rupture of the Sinus of Valsalva	+	+	Does not apply	Continuous murmur	-	+	No change	Turbulent flow	Saccular aneurysm		Emergent surgery needed, more over after MI
Fistulas of the Coronary Circulation	+	+	Does not apply	Continuous murmur	-	+	Unremarkable / Cardiomegaly	Not applied	Suggestive markers	Coronary artery Angiography

Differentiating Patent Ductus Arteriosus from other Diseases

Venous Hum

• Frequently heard in children over the base of the neck, usually best on the right side.
• Changes with position. Disappears in the supine position or with compression.
• Louder in diastole

Mammary Souffle

• Heard during late pregnancy and the early postpartum period in lactating women.
• Thought to be arterial in origin
• Can be bilateral
• Is louder, peaks in systole
• Vanishes in the upright position
• Abolishes by local compression

Aortopulmonary Window

• It's a rare congenital opening between the aorta and the pulmonary trunk just above the aortic valve.
• It can be associated with other abnormalities like anomalous origin of the coronary arteries from the pulmonary trunk and coarctation of the aorta.
• The murmur is lower and more medial in location.
• In adults is presented without a murmur and clinical features of the Eisenmenger's syndrome.

Rupture of the Sinus of Valsalva

• It can rupture into a cardiac chamber. Almost always arise from the right or the noncoronary cusps and rupture into the RV and RA respectively. Occasionally is acquired as a result of endocarditis. Large acute perforations tend to occur between puberty and age 30 causing severe retrosternal chest pain, dyspnea related to the large left-to-right shunt. The murmur is louder in a lower parasternal position. People with VSDs and sudden development of chest pain have frequently experienced rupture of a coexistent sinus of valsalva aneurysm. A rupture of the sinus of valsalva can distort or compress the coronary arteries and cause an infarction, distort the conduction system, cause AV block, distort the aortic valve, and cause AS or AI. Patients with rupture of the sinus of valsalva, should undergo surgical correction because mortality is high within a year of rupture.

Fistulas of the Coronary Circulation

• Generally a coronary artery that arises normally will communicate with the RV.
• Occasionally drain into the pulmonary trunk.
• The artery that forms the fistula is generally dilated, elongated, and tortuous. The left-to-right shunt is small.
• It may not be recognized radiographically.
• Patients with small fistula are generally asymptomatic. Therefore, no justification to repair it.
• On the other hand, if the shunt is extremely large, then failure may develop in the 4th, 5th or 6th decade of life. It can be treated with ligation.

Anomalous Origin of the Coronary Artery from the Pulmonary Trunk

• Usually refers to the origin of the left coronary artery from the pulmonary trunk.
• Approximately, 80 to 90% of the patients die in their first year of life due to ischemia.
• Blood from the high pressure RCA flows to the low pressure left coronary artery and the pulmonary artery.
• Anomalous origin of the RCA from the PA is much rarer, but these patients stand a better chance of surviving into adulthood because it is less likely to cause ischemia early in life.

Pulmonary Arteriovenous Fistula

• Instead of being localized to the precordium, these murmurs are localized to the lung fields. Cyanosis is presented with a normal heart size. Seen in Rendu-Osler-Weber syndrome. A fistula causing cyanosis could be treated with lobectomy if it is confined to a single lobe.