FINAL SCRIPT

Slide 1: Cover slide

Good Morning, Doctors. I am ______________,.Together with my
colleagues, Apple Cabuga, Claudine Geraldo and Joseph Guadalupe, or Glynne Pulmon

Slide 2: History
Presenting to you a case of a 30 year old female patient who was well until 5 weeks prior to
admission when she felt pain in her left flank, hip and thigh. Two weeks prior to admission, she
then developed progressive dyspnea and dry cough.

Slide 3: Transfer
She was then admitted to another hospital for two days where she was examined and
underwent radiographic studies and laboratory tests. She was consequently transferred to this
hospital on her third day of admission.

Slide 4: New Hospital

Upon transfer, a thorough cardiac examination was conducted on the patient. Various laboratory
tests, radiographic and other imaging studies as well as several procedures were
performed.Medications as well as crucial interventions were utilized during admission.

Slide 5: Hospital Stay

The patient was then admitted to the Respiratory Intensive Care Unit, where she was closely
monitored. Key interventions and management were executed until her seventh hospital day
where a lung biopsy was performed.

Slide 6: Salient Features

Slow Down!!!!!
Based on the case, our salient features are the following:
Progressive dyspnea
Dry cough
Left flank, hip and thigh pain
Tachypnea
Tachycardia
Elevated Blood pressure
Low grade Fever
Jugular venous distention
 Wide splitting of S2
Respiratory alkalosis
Hypoxemia
Inspiratory crackles
Right ventricular lift
Cardiac Enlargement
Long standing Murmur
Filling defects
Pulmonary edema
Round nodular densities
Prominent RV & pulmonary artery
Arterial hypertension

Prior to considering the pivot of this case, we considered the different laboratory values
such as:

• Decreased hematocrit
• Proteinuria
• Leukocytosis
• Neutrophilia
• Lymphocytopenia
• Thrombocytopenia
• Increased ESR
• Increased glucose
• Hypoalbuminemia
• Increased LDH
• Increased creatine phosphokinase
• Increased amylase
• Increased PT, APTT and thrombin
• Decreased urea nitrogen
• Increased bilirubin

Slide 7 : Pivot

Among the following salient features, we have tapered it down to the pivot of this case.
Progressive dyspnea

Slide 8: Progressive Dyspnea

A concencus from the American Thoracic Society defines dyspnea as a term used to
characterize a subjective experience of breathing discomfort that is comprised of qualitatively
distinct sensations that vary in intensity. The experience derives from interactions among
multiple physiological, psychological, social, and environmental factors, and may induce
secondary physiological and behavioral responses.

A statement from the American Thoracic Society stipulates that Dyspnea is considered acute
when it develops over hours to days and chronic when it occurs for more than four to eight
weeks. Some patients present with acute worsening of chronic breathlessness that may be
caused by a new problem or a worsening of the underlying disease such as asthma, chronic
obstructive pulmonary disease and even heart failure.

Slide 9: Conditions & Causes (dyspnea)

According to the American Academy of family physicians, dyspnea can be classified into four
categories: non-cardiopulmonary,Pulmonary, mixed cardiopulmonary, Cardiac. Several
conditions under these different classifications are presented below.

Arrhythmia

Slide 10: Differential Diagnosis

From all these given causes of dyspnea we chose the conditions that would most likely be
clinically relevant with our case.

So, under the non cardiopumonary cause , we picked Gastroesophageal Disorder

Pulmonary causes we chose both interstitial pulmonary fibrosis as well as acute respiratory
distress syndrome.

Next, for the mixed classification, we picked Chronic Pulmonary Emboli.

Lastly, under the cardiac category of dyspnea, among the following we chose atrial septal
defect.

From these various categories, These following conditions point to our various differential
diagnoses.

Slide: Gastroesophageal Reflux Disease

First on our list of differentials is GastroEsophageal Reflux Disorder.

Slide: Definition
Gastroesophageal reflux disease (GERD)as defined by the American College Of
Gastroenterology is a chronic medical condition caused by the flow of contents from
the stomach upwards into the esophagus resulting in both symptoms and
complications.
Slide: Etiology

Currently, there is no known cause to explain the development of GERD. Over the
years, several risk factors have been identified and implicated in the pathogenesis of
GERD. Motor abnormalities such as esophageal dysmotility causing impaired
esophageal acid clearance, impairment in the tone of the lower esophageal sphincter
(LES), transient LES relaxation, and delayed gastric emptying are included in the
causation of GERD.

Several other risk factors have been independently associated with the development of
GERD symptoms that include age ≥50 years, low socioeconomic status, tobacco use,
consumption of excess alcohol, connective tissue disorders, pregnancy, postprandial
supination, and different classes of drugs which include anticholinergic drugs,
benzodiazepines, NSAID or aspirin use, nitroglycerin, albuterol, calcium channel
blockers, antidepressants, and glucagon

Slide: Epidemiology

GERD is one of the most common gastrointestinal diseases. It is thought that up to 20%
of the US population has GERD. Almost everyone will experience heartburn at some
point, especially after a heavy meal. However, GERD is defined as frequent symptoms
(two or more times a week) or when the esophagus suffers damage from reflux such as
narrowing, erosions, or pre-cancerous changes. GERD is more common amongst the
elderly, obese and pregnant women.

Slide: Pathophysiology

The pathophysiology of GERD is complex. The antireflux barrier is created by a combination of

the normal anatomical configuration of the esophagogastric junction and the strength and
function of the lower esophageal sphincter. A weak antireflux barrier causes reflux in the
majority of patients. Gastroesophageal reflux disease most often occurs when LES pressure is
low or the normal angulation of the EG junction is lost. Physiologic reflux is generally brief in
duration, relatively infrequent, and occurs almost exclusively after meals and is caused by a
sudden relaxation of the LES that is not induced by swallowing.

Slide:Clinical Manifestations
The most common symptoms of GERD are heartburn and regurgitation. Heartburn is a
burning sensation in the chest behind the breastbone. Regurgitation is a feeling of fluid
or food coming up into the chest. Many people experience both symptoms; however,
some patients can have one without the other.

Slide: Laboratory & Finding

Aside from a detailed history and physical exam of the patient, diagnostic tests ordered are the
following:

1. Upper Endoscopy
2. Ambulatory pH probe test
3. X-ray of the upper digestive system
4. Esophageal manometry
5. Transnasal esophagoscopy

Slide: Rule in & rule out

The following findings were useful in considering this condition but due to these following
reasons, it was then ruled out.

RULE IN RULE OUT

Dyspnea (-) dysphagia

Dry cough (-) Chest pain

Abdominal distention No upper endoscopic exam done

Leukocytosis

Slide: Acute Respiratory Distress Syndrome

Another differential diagnosis we considered is Acute respiratory distress syndrome or

ARDS.

Slide:Definition
The American Lung Association defines Acute respiratory distress
syndrome (ARDS) as a clinical syndrome of severe dyspnea of rapid onset,
hypoxemia, and diffuse pulmonary infiltrates leading to respiratory failure.
Slide:Etiology

Most cases, around (>80%) are caused by a relatively small number of

clinical disorders with pneumonia and sepsis estimated at
~40–60%.Followed in incidence by aspiration of gastric contents, trauma,
multiple transfusions, and drug overdose
Slide:Epidemiology

Epidemiologic studies have shown that ALI/ARDS is more common and

has a worse prognosis in chronic alcohol- ics and in smokers.

In a 2016 study of intensive care units in 50 countries, the incidence of

ARDS was 10.4%, and mortality rates were 35% for mild, 40% for
moderate, and 46% for severe ARDS. The majority of deaths are
attributable to sepsis, multiorgan failure, or severe lung injury.

Slide:Pathophysiology

The natural history of ARDS is marked by three phases—exudative,

proliferative, and fibrotic—

○ Exudative Phase In this phase, alveolar capillary endothelial

cells and type I pneumocytes (alveolar epithelial cells) are
injured, with consequent loss of the normally tight alveolar
barrier to fluid and macromolecules. Collapse of large sections
of dependent lung can contribute to decreased lung
compliance. Consequently, intrapulmonary shunting and
hypoxemia develop and the work of breathing increases,
leading to dyspnea. The pathophysiologic alterations in
alveolar spaces are exacerbated by microvascular occlusion
that results in reductions in pulmonary arterial blood flow to
ventilated portions of the lung (and thus in increased dead
 space) and in pulmonary hypertension. Thus, in addition to
severe hypoxemia, hypercapnia secondary to an increase in
pulmonary dead space can be prominent in early ARDS.The
exudative phase encompasses the first 7 days of illness after
exposure to a precipitating ARDS risk factor, with the patient
experiencing the onset of respiratory symptoms. Although
usually presenting within 12–36 h after the initial insult,
symptoms can be delayed by 5–7 days. Dyspnea develops,
with a sensation of rapid shallow breathing and an inability
to get enough air.

○ Proliferative Phase This phase of ARDS usually lasts from

approximately day 7 to day 21. Many patients recover rapidly
and are liberated from mechanical ventilation during this phase.
Despite this improvement, many patients still experience
dyspnea, tachypnea, and hypoxemia.

○ Fibrotic Phase While many patients with ARDS recover lung

function 3–4 weeks after the initial pulmonary injury, some enter
a fibrotic phase that may require long-term support on
mechanical ventilators and/or supplemental oxygen. The
physiologic consequences include an increased risk of
pneumothorax, reductions in lung compliance, and increased
pulmonary dead space. Patients in this late phase experience a
substantial burden of excess morbidity. Lung biopsy evidence
for pulmonary fibrosis in any phase of ARDS is associated with
increased mortality risk.

Slide: Laboratory or Imaging Tests

1.CHEST XRAY
2. CT SCAN
Slide: Diagnostic Criteria

In diagnosing acute respiratory distress syndrome, a diagnostic criteria must be met based on
the various categories including severity of oxygenation, onset, chest radiograph and the
absence of left atrial hypertension.

○
Slide: Rule in & Rule out

With pertinent findings and information from the case, we considered this condition due to these
following findings but was then ruled out due to the succeeding reasons.

Rule In Rule Out

○ Hypoxemia ○ Left flank, hip and thigh

○ Dyspnea pain
○ Tachypnea ○ Elevated blood pressure
○ Pulmonary edema ○ Jugular venous
○ Dry cough distention
○ Respiratory alkalosis ○ Wide splitting of S2
○ Tachycardia ○ Inspiratory crackles
○ Low grade fever ○ Right ventricular lift
○ Cardiac enlargement
○ Long standing murmur
○ Filling defects
○ Round nodular densities

Slide: Interstitial Pulmonary Fibrosis

Interstitial Pulmonary Fibrosis is also one of the differential diagnoses that we considered.
Slide:Definition
● IPF is classified as a form of idiopathic interstitial pneumonia, which is a group of lung
diseases that damage the lungs in a similar manner and occur due to unknown causes.
Interstitial lung disease (ILD), sometimes called diffused parenchymal diseases, describes
a heterogeneous collection of distinctive lung disorders classified on the grounds of
shared clinical, radiographic, physiologic or pathologic factors.
● Diffuse parenchymal lung diseases include a large number of heterogeneous conditions
that affect the lung parenchyma with varying degrees of inflammation and fibrosis.
● Idiopathic pulmonary fibrosis (IPF) is a lung disorder where there is scarring of the lungs
from an unknown cause. It is usually a progressive disease with a poor long-term
prognosis.

Slide:Etiology
○ The exact etiology for the development of IPF is unknown, but risk factors like exposure
to tobacco smoke, metal, wood, dust, and gastroesophageal reflux have been implicated.
The current theory on the etiology of IPF is that recurrent injury to the alveolar
epithelium triggers a cascade of signaling by the immune system leading to fibrosis. A
dysregulated response to the injury can cause tissue remodeling.

Slide:Epidemiology
○ IPF usually presents after the fifth or sixth decade of life and is more common with older
age. There is a global distribution, and the incidence appears to be increasing.
Slide:Pathophysiology
○ Environmental factors like smoking, chronic aspiration, or viral infections along with
advancing age can lead to respiratory alveolar epithelial injury and are thought to be the
likely driving factors for the pathogenesis of IPF. With an epithelial injury, there is an
activation of fibroblasts and dysregulated repair of the alveolar epithelium. When this
leads to increased matrix deposition in the lung interstitium and scarring, there is a
destruction of lung architecture that results in pulmonary fibrosis.[7] The destruction of
lung architecture impairs gas exchange and will progress to hypoxic respiratory failure, a
hallmark of advanced disease.
Slide: Clinical Manifestations
○ The most common presenting symptoms of IPF are dyspnea on exertion and cough,
followed by fatigue. The diagnosis is often delayed, as most patients are diagnosed more
than a year after symptom onset. Since symptoms are nonspecific and the disease is
idiopathic, other conditions must be excluded before diagnosis. History of exposure to
inhaled dust, metals, asbestos, mold, or birds, should be elicited to exclude other
interstitial lung diseases. Medication and drug history is important to exclude drug
toxicities. A careful history should be elicited to exclude autoimmune conditions like
rheumatoid arthritis, scleroderma, Sjogren disease, or poly/dermatomyositis should also
 be elicited since these conditions can all lead to interstitial lung disease. The cough is
most commonly dry and non-productive.
○ The physical exam should be focused on evaluating lung involvement, extent and
severity of the disease, and excluding another diagnosis. The classic pulmonary exam
usually reveals fine bibasilar “velcro” crackles during inspiration.
Slide: Laboratory or Imaging findings
1. Pulmonary Function
2. Chest Imaging
Slide: Diagnostic Criteria

Diagnostic criteria includes a major as well as a minor criteria.

Major criteria includes:

1. Exclusion of other known cause of ILD
2. Abnormal Pulmonary function studies
3. Bibasilar abnormalities
4. Transbronchial lung biopsy

Minor criteria includes:

1. Age greater than 50 years of age
2. Insidious onset of otherwise unexplained dyspnea
3. Duration of Illness > 3 months
4. And bibasilar inflammatory crackles.

Slide: Rule in & Rule out

This condition was ruled in due to these salient features such as dyspnea, cough and inspiratory crackles
but was then ruled out due to the following reasons.

Rule in Rule out

● Dyspnea ● No history of risk factors

● Cough ● Age group (common 5th-6th decade)
● Inspiratory crackles on the base ● IPF have an abnormal chest radiograph at
● Tachypnea the time of presentation
● Tachycardia ● Peripheral reticular opacities are usually
● Cyanosis most notable in the lower lobes
● Hypoxemia ● No pulmonary function test
● Increased ESR ● No clubbing
● Increased LDH ● Left flank, hip and thigh pain
● Cardiac enlargement
● Long standing murmur
● Filling defects
Slide: Pulmonary Emboli

Next on the list of differentials, is Pulmonary Embolism.

Slide:Definition

Pulmonary embolism (PE) occurs when there is a disruption to the flow of blood in the pulmonary artery
or its branches by a thrombus that originated somewhere else. Pulmonary vascular occlusion occurs and
impairs gas exchange and circulation. In the lungs, the lower lobes are more frequently affected than the
upper, with bilateral lung involvement being common.

Slide:Etiology
○ Virchow's triad of hypercoagulability, venous stasis, and endothelial injury provides an
understanding of these risk factors.
○ A study by Porembskaya et.al stated that DVT was generally acceptable as the cause of a
pulmonary emboli, as well as an emboli without a primary cause. But with technological
advancement, thrombotic masses in the pulmonary artery is now an emerging cause.

Slide:Epidemiology

The incidence of pulmonary embolism (PE) ranges from 39 to 115 per 100 000 population
annually. After coronary artery disease and stroke, acute pulmonary embolism is the third most
common type of cardiovascular disease.

The incidence of PE is noted to be more in males as compared to that in females.

Slide:Pathophysiology

The most common gas exchange abnormalities are arterial hypoxemia and an increased alveolar-arterial
O2 tension gradient, which represents the inefficiency of O2 transfer across the lungs. Anatomic dead
space increases because breathed gas does not enter gas exchange units of the lung.

Slide: Clinical Manifestations

Clinical Manifestations of pulmonary embolism involve unexplained breathlessness, pleuritic

chest pain, cough, hemoptysis.

Dyspnea may be acute and severe in central PE, whereas it is often mild and transient in small peripheral
PE. While in patients with preexisting heart failure or pulmonary disease, worsening dyspnea may be the
only symptom.
Slide: Laboratory or Imaging findings

Laboratory or imaging findings commonly ordered that is deemed useful are:

1. Arterial Blood Gas

2. D-Dimer
3. Electrocardiography
4. Chest Xray
5. Pulmonary Angiography
Slide: Diagnostic Criteria

In diagnosing pulmonary emboli, several diagnostic criteria must be met. Shown here are the
Clinical Decision rules and the revised geneva score that is useful in the diagnosis of Pulmonary
embolism.

Slide: Rule in & Rule out

When considering this diagnosis, these are the different findings which made us rule in
Pulmonary Emboli.

● Progressive dyspnea
● Dry cough
● Left flank, hip and thigh pain
● Tachypnea
● Tachycardia
● Elevated Blood pressure
● Fever
● Jugular venous distention
 ● Wide splitting of S2
● Respiratory alkalosis
● Hypoxemia
● Inspiratory crackles
● Filling defects
● Pulmonary edema
● Round nodular densities

Slide 12: Atrial Septal Defect

The American Heart Association defines Atrial septal defect as a hole in the wall or septum
that separates the upper chambers of the heart which is the atria.

This defect allows oxygen-rich blood to leak into the oxygen-poor blood chambers in the
heart. ASD is a defect in the septum between the heart's two upper chambers. The septum is a
wall that separates the heart's left and right sides.

Slide:Etiology

For the etiology, Atrial septal Defects are caused by Mendelian inheritance, aneuploidy,
transcription errors, mutations, and maternal exposures. Atrial septal defects are noted in patients
with Down syndrome, Treacher-Collins syndrome, Also, Maternal exposure to rubella and drugs,
such as cocaine and alcohol can also predispose the unborn fetus to develop an ASD.

Slide:Epidemiology

Children with ASDs usually either are asymptomatic or suffer only mild exertional
dyspnea. The resultant increased pulmonary blood flow, right heart overload,
arrhythmias, and pulmonary hypertension tend to increase with age.

Atrial septal defect (ASD) is the most prevalent congenital cardiac anomaly in adults,
accounting for ~35% of all congenital heart defects. Late presentation is due to the
insidious development of right ventricular remodeling, with enlargement of right cardiac
chambers.

Slide:Pathophysiology

During fetal development, the upper chambers of the heart have an opening which allows the
blood to bypass lungs by passing directly to the left atrium.After birth, the opening closes or
becomes smaller in several weeks or months. However, in an individual with ASD this closure
did not occur.

Small atrial septal defects might be found by chance and never cause a concern. Others close
during infancy or early childhood.A large, long-term atrial septal defect can damage the heart
and lungs. Surgery may be needed to repair an atrial septal defect and to prevent complications

Slide: Clinical Manifestations

Very small ASDs with a diameter <5 mm) may not have significant clinical consequences, while
a defect of 5–10 mm may lead to symptoms in the fourth or fifth decade of life. Larger defects
generally >10 mm typically present with symptoms in the third decade of life.

When symptoms occur, patients often first notice dyspnea, fatigue, exercise intolerance, or
palpitations. Some patients may present with syncope or even with peripheral edema from overt
right heart failure and others may develop recurrent pulmonary infections.

Physical Exam Finding would reveal the following:

1. Right ventricular heave
2. Systolic Flow murmur in the pulmonary valve region due to increased pulmonary flow
3. Fixed Split-second heart sound

Slide: Diagnostic Criteria

Diagnostic tests useful in diagnosing this condition are the following:

1. Echocardiogram
2. Chest X-ray
3. Electrocardiogram
4. MRI scan
5. CT scan

Slide: Rule in & Rule out

We considered atrial septal defect as a diagnosis due to the following findings in this case.

Rule in Rule out

Long standing murmur

Widely Split S2

Cardiac enlargement
 Prominent main pulmonary artery

Prominent pulmonary vasculature

Cyanosis

Progressive dyspnea

Right ventricular lift

APPROACH TO DIAGNOSIS

Long term effects of Atrial septal defect in Adulthood would cause the reversal of the left
to right shunting of the heart to a right to left shunt that would cause the otherwise
asymptomatic condition to become symptomatic. This could manifest with long standing
cardiac murmurs , right axis deviation, incomplete bundle branch block, right ventricular
hypertrophy and the thickening of the cardiac wall. These findings and manifestations all
point to an atrial septal defect. This phenomenon would then cause a cascade of
complications that if not managed or treated could become fatal.

One of its most common and serious complications is Pulmonary Hypertension.

Harrison’s Internal Medicine defines Pulmonary hypertension as a heterogeneous
disease involving pathogenic remodeling of the pulmonary vasculature, which increases
pulmonary artery pressure and vascular resistance.

A recent study by Levine et.al concluded that PAH is a rare disorder found in 15 to 50
persons per million within the United States and Europe.Generally, PAH affects women
aged between 30 and 60 years.

Clinical findings of this disease includes a right ventricular lift, inspiration with s2
splitting,increased intracardiac pressures, prominent pulmonary artery, dry cough,
tachypnea and tachycardia.

The origin of Pulmonary hypertension varies depending on the group or classification of

the pulmonary hypertension.
Group 1: Pulmonary arterial Hypertension refers to increased pressure in the vessels caused by
obstruction in the small arteries in the lung,

Group 2: Pulmonary hypertension due to left-side heart disease. This is when long-term heart
disease results in damage to the pulmonary arteries eventually causing Pulmonary hypertension.

Group 3: Pulmonary hypertension caused by underlying lung diseases or hypoxemia. The

common diseases that cause hypoxia.

Group 4: CTEPH (chronic thromboembolic pulmonary hypertension)—In some patients,

pulmonary embolism, or blood clots, in the lung’s arteries can form scar-like tissue, blocking or
narrowing the arteries and leading to CTEPH.

Group 5: Pulmonary hypertension from numerous other disorders. This group includes any
other cause that doesn’t fit under another heading.

Through constant remodeling of the pulmonary vasculature, in time would cause mean
pulmonary arterial pressure to exceed more than 25 mm Hg at rest or greater than 30
mm Hg during exercise. A progressive and sustained increase in pulmonary vascular
resistance that eventually may lead to right ventricular failure. To preserve cardiac
output during elevated right ventricular afterload, right ventricular work must increase. A
sustained or sometimes progressive increase in right ventricular work causes a shift in
the efficiency of right ventricular systolic function.

This persistent increase in pulmonary pressure acting on a remodeled vessel could

become serious. Taking into consideration the virchow’s triad, the increased pressure
could cause endothelial dysfunction and eventually could cause injury. In the attempt to
heal the damaged vessel, thrombus formation would likely form and seal the damaged
vessel.

But in the presence of a remodeled blood vessel, a thrombus formation could be fatal. A
remodeled vessel plus the thrombus formation could cause further narrowing of the
blood vessel. The increased pressure on this said artery could eventually cause the
formed thrombus to dislodge, becoming an emboli. As the thrombus becomes an
emboli, this could present with progressive dyspnea, cough, cyanosis, hypoxemia,
jugular vein distention, tachypnea, tachycardia and an elevated blood pressure. This
emboli could then be lodged into the smaller vessels of the lungs causing life
threatening complications.

Slide: Summary

This is a case of a 30 year old female patient with an underlying atrial septal defect as
evidenced by the clinical manifestations and imaging studies
Patient also manifested with pulmonary hypertension upon cardiac catheterization.

Pulmonary emboli was also revealed by imaging studies.

Hospital management included TPA’s, antithrombotics, vasodilators, vasoconstrictors,

and antibiotics. She was also given 100% oxygen through mechanical ventilation
followed by hypothermia and ECMO soon after.

The patient’s condition was noted to improve a little.

At the 9th Hospital day, X-ray & BOPA revealed round nodular densities suggestive of a
pulmonary emboli.

This brings us to our final diagnosis

Slide: Final

Pulmonary Embolism secondary to Pulmonary Hypertension from an underlying atrial

septal defect.

Thank you, Good Morning and God Bless!

END!!!!!!!