Patent Ductus Arteriosus

Paul Scavella
4th Year Medical Student
Lecturer: Dr. Carlos Thomas
University of the West Indies, SCMR

Outline
Definition
Epidemiology
Anatomy & Embyrology
Aetiology
Pathophysiology
Clinical Presentation
Differential Diagnosis
Evaluation

Definition
Congenital Heart defect
Ductus arteriosus fails to close after birth
> 3 months in full term infant = Persistent Patent
Ductus arteriosus

Epidemiology
Estimated incidence in US (term infants) = 0.006% - 0.02%
Incidence increases in preterm babies

20% = > 32 weeks gestation

Up to 60% = < 28 weeks gestation

Up Up to 30% of low birth weight (<2500g) babies develop PDA

Siblings of those who have had are at increased risk (5%

recurrence rate
Isolated PDA represents 5 10% of all congenital heart defects
There is a female preponderance (2:1 or 3:1)

Anatomy & Embryology

Normal structure during fetal life

Allows most of the blood leaving the right ventricle to

bypass the pulmonary circulation

Remnant of 6th aortic arch, connects pulmonary artery

to the descending aorta
Anatomical landmark = Left Recurrent laryngeal nerve
Typically left aortic remnant; however it can be rightsided or on both sides

Anatomy

Anatomy: Flow chart of Fetal Circulation

Anatomy

Anatomy & Embryology

Classified by the
Krichenko classification
anatomically
Type A (Conical)

Type B (Window)

Type C (Tubular)

Type D (Complex)

Type E (Elongated)

Aetiology
Sporadic
Multifactorial

Genetic + Environmental

Aetiology
Genetic Foactors

Chromosomal abberations: Trisomy 21

Single gene mutations: HoH-Oram Syndrome/Char

Syndrome (TPAP2B mutations)

X-linked mutations

Familial cases
Teratogens
Prematurity/Immaturity

Aetiology contd
Factors that increase incidence

Prematurity
Inversely related to gestational age
~ 45% of infants < 1750 grams
~ 80% of infants < 1000 grams

RDS: PDA correlated with severity or RDS. After surfactant

there is increased risk of clinically symptomatic PDA

Fluid overload

Asphyxia

Aetiology contd
Factors that decrease incidence

Intrauterine Growth Restriction

Antenatal steroid administration

Prolonged rupture of membranes

Pathophysiology
The patent ductus allows the shunting of blood from the high pressure aorta to the
low pressure pulmonary artery, increasing the volume of blood passing through
the lungs and returning to the left atrium.
This is similar to an increased preload and leads to left atrial dilation, increased LA
pressure, increased PV pressure and ultimately pulmonary congestion (left-sided
congestive heart failure).
Bulging of the aorta and pulmonary artery proximal to the PDA occurs as a result
of increased blood volume and turbulent flow.
There is always a pressure difference between the aorta and pulmonary artery
(greatest during systole), and consequently continuous flow through the PDA
producing the characteristic continuous murmur.
The increased flow through the pulmonary artery can result in pulmonary
hypertension. When the pressure in the pulmonary artery equals or even exceeds
that of the aorta, either the diastolic portion of the murmur or the complete
murmur may disappear due to flow reversal (reverse shunting PDA). Blood then
bypasses the lungs and the patient presents with cyanosis and a compensatory
polycythaemia.

Clinical Presentation
Small PDA

Qp/Qs < 1.5

Moderate PDA

Qp/Qs = 1.5 2.2

Large PDA

Qp/Qs > 2.2

Clinical Presentation: History

Small PDAs = Asymptomatic; almost undetectable flow
shunting

Incidental finding

Moderate PDAs = Compensate well through childhood

May remain asymptomatic into adulthood

Symptomatic in 3rd decade of life

Exercise intolerance

Large PDAs =

Clinical Presentation: History

Clinical Presentation: Physical Examination

Physically underdevelopment due to large shunt
Maternal rubella syndrome
Rocker bottom feet in trisomy 21

Clinical Presentation: Physical Examination

Clinical Presentation: Physical Examination

Clinical Presentation: Physical Examination

Differential cyanosis (DC) and clubbing present in

shunt reversal

DC can be brought on by exercise or warm water bath

Useful to have patient sit with hands and feet together

JVP normal in small shunts

Patients w/cardiac failure = JVP elevated with prominent A

& V waves

Patients w/pulmonary hypertension = prominent A waves

Clinical Presentation: Physical Examination

Clinical Presentation: Physical Examination

Arterial pulse

Wide pulse pressure

Brisk rise, single peak and rapid collapse

Diastolic pressure is low, systolic high

Peripheral pulses bounding

If shunt is small or pulmonary hypertension present
bounding pulse is absent

Palpable thrill and P2 (Pulmonary hypertension)

Clinical Presentation: Physical Examination

Arterial pulse

Wide pulse pressure

Brisk rise, single peak and rapid collapse

Diastolic pressure is low, systolic high

Peripheral pulses bounding

If shunt is small or pulmonary hypertension present
bounding pulse is absent

Palpable thrill and P2 (Pulmonary hypertension)

Clinical Presentation: Physical Examination

Auscultation

Classic continuous machinery murmur

Continues throughtout S1 and S2

Left upper sternal border or left infraclavicular area

Grade 2 3 in small PDA; 4 in moderate to large PDA

S1, S2 normal with an accentuated P2

S3 diastolic rumble: moderate large PDA

Peaks at S2 and declines in intensity in diastole

Differential Diagnoses
Coronary arteriovenous fistula.
Systemic arteriovenous fistula.
Pulmonary arteriovenous fistula.
Venous hum.
Tetralogy of Fallot (with absent pulmonary valve)
Ruptured aneurysm of the sinus of Valsalva (seen in Marfan's
syndrome).
Aortopulmonary septal defect (aortopulmonary window).

Investigations

Chest X ray
Normal Cardiomegaly and increased pulmonary
vasculature

Transthoracic echocardiogram = Gold Standard

Assessment of ductal size, geometry, degree of the
shunt and pulmonary pressures

ECG
Left Atrial Enlargement
Right Ventricular Hypertrophy

Left Ventricular Hypertrophy

Treatment

Clinical Presentation
Abdominal pain may present with varying characteristics:

Persistent or intermittent

Waxing and waning or stead and unrelenting

Sharp or dull

Worsened or unaffected by movement

Associated complaints including:

Vomiting

Diarrhoea

Constipation

Fever

Weight loss

Headace

Anorexia

Differential Diagnosis
Life-Threatening

Other

Trauma

Constipation

Appendicitis

Gastrointestinal infection, Peptic

Ulcers

Intussusception

UTI

Malrotation with midgut volvulus

Streptococcal pharyngitis

Incarcerated inguinal hernia

PID

Intestinal obstruction

Mesenteric lymphadenitis

Necrotizing enterocolitis

Ruptured ovarian cyst

Foreign body ingestion
Colic

Differential Diagnosis contd

Functional Abdominal Pain

Most children with Recurrent Abdominal Pain (RAP) = Functional

abdominal pain

Functional Gastrointestinal Disorders can be subdivided into 5

categories:

Functional Abdominal Pain (FAP), FAP Syndrome, Functional

dyspepsia, Irritable bowel syndrome and Abdominal migraine

Evaluation
History
Physical Examination
Investigations
Treatment

Evaluation: History

Evaluation: History contd

Other details

Bowel movement patterns and stool quality (size, hard, soft,

amount, odour)

Ingest of toxin or foreign object

Accidental or non-accidental trauma

Dietary history: Milk Constipation in young children

Past Medical History

CF, Spina bifida/Cerebral Palsy/Developmental delay, Sickle cell
disease, recurrent respiratory tract infections

Sexual history screen for STI

Females: Dont forget about Menstrual cycles (menarche,
regularity, amount of bleeding, relation to abdominal pain)

Family History Inflammatory Bowel Disease

Travel, Social and Psychiatric (potential stressors) history

Evaluation: Physical
Examination
ABCs
Vitals
Growth Parameters (is there evidence of failure to thrive?)
Inspection
Auscultation
Palpation
Percussion

Evaluation: Physical Examination contd

Inspection

Contour, Symmetry

Pulsations, visible peristalsis

Vascular irregularities

Skin markings

Wall protrustions

Signs of trauma (bruising, swelling)

Abdominal distension

 Auscultation

Bowel sounds (Hyperactive, Normoactive, Hypoactive or Absent)

Abdominal bruits
Hepatic Hum (Portal hypertension)
Splenic rub (Splenic infarction

Abdominal Aorta or renal bruits

While auscultating test for tenderness

Evaluation: Physical Examination contd

Palpation

Tenderness on light and deep palpation, identify region(s)

Abdominal masses (including fecal mass)

Guarding and rebound tenderness

Liver and spleen

While there although percussion we generally percuss for liver
span and splenic tip

Ballot both kidneys

Evaluation: Physical Examination contd

Percussion

Assess general tone (Tympanic vs. non-tympanic)

Assess for ascites

Shifting dullness

Fluid thrills

Digital Rectal Examination

Examine the anus for fissures and skin tags

Tone

Stool and blood

Special circumstances

Females may need a genital assessment

Evaluation: Investigations
In general laboratory tests include:

A CBC, Urine dipstick and Urinalysis if abnormal and ESR (in

recurrent cases)

Serum chemistries

Urine pregnancy testing (Beta human chorionic gonadotropin

hormone) postmenarche

Lactose intolerance = hydrogen breath test

Dietary manipulation, or exogenous lactase (lactaid) may be more

cost effective

Stool: H. pylori Antigen test, Ova and parasites and fecal

occult testing

Evaluation: Investigations
contd
Imaging includes

Abdominal and chest films

Ultrasound

CT scans

Plain radiographs demonstrate signs of obstruction or perforation

Ultrasonography preferred modality for:

Gallstones

Genitourinary conditions such as ovarian torsion, ruptured ovarian cyst

and testicular torsion

CT with contrast

Appendicitis, Pancreatitis, Abscess, Mass

Endoscopy for those referred to Gastroenterologist

Treatment
Medical Conditions = Pharmacological agents

Peptic Ulcer disease H2 blockers (cimetidine), PPIs

(omeprazole) or cytoprotective agents (sucralfate)

Infection with H. pylori Combo of antibiotics

(amoxicillin/metronidazole, Clarithromycin), PPIs and bismuth
preparations

UTIs Antibiotics based on sensitivity

Surgical Conditions = Surgical intervention

Appendicitis, Necrotizing enterocolitis, malrotation w/midgut

volvulus

RAP: Treatment
3 tiered empiric trial
High fiber diet
Antacids, H2 blockers, Proton Pump Inhibitors
A trial of lactaid or lactase

Low dose Tricyclic Antidepressants (eg. Amitriptyline 0.1mg/kg

0.2mg/kg) has been used successfully in children with
functional abdominal pain

MCQs
A 24 year old dress designer complains of a crampy periumbilical pain.
These symptoms have been present over the past 9 months since she
has began her first job after graduating art school. During that time,
she has had several episodes of constipation lasting 4-5 days typically
followed by 3-4 days of frequent loose bowel movements. She denies
any blood in her stools, fever, weight loss or change in appetite. Her
symptoms are generally milder on weekends. Her physical
examination is normal. Her WBC is 6700/mm 3, her hematocrit 38% and
her ESR 4mm/h Her serum albumin and liver function tests are normal.
Which of the following is the most likely diagnosis?
a. Crohns Disease
b. Diverticulosis
c. Giardia infection
d. Irritable Bowel Syndrome
e. Ulcerative colitis

MCQs
All of the following are correct except:
a. Urine Beta hCG is done routinely on females with
abdominal pain
b. CT is useful in diagnosing equivocal appendicitis
c. Chest X-ray is useful in evaluating abdominal pain
d. Ultrasound is the preferred modality for diagnosing
gallstones on imaging