Failure to Diagnose & Delay in Treatment of Pediatric Pulmonary Hypertension

Evaluate breaches in the standard of care by asking and answering the questions below and analyzing the results to determine whether or not there was a Failure to Diagnose & Delay in Treatment of Pediatric Pulmonary Hypertension

Answer the following questions to find out if the standard of care was followed for your case.

  • With which of the following symptoms of pulmonary hypertension did the patient present? (Choose all that apply)
    Not elicited
    Answer unknown
    Shortness of breath
    Exercise intolerance
    Easy fatigability
    Chest pain
    Persistent cough
    Syncope/dizziness
    Irritability/poor feeding
    Cyanotic episodes(bluish or purplish discoloration of the skin)
    Swelling of legs/abdomen
    None of the above
    Not elicited
    Answer unknown
    Why is this important?
    The pediatric patient with pulmonary hypertension may present with symptoms including shortness of breath, exercise intolerance and easy fatigability.
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  • Which of the following physical signs were observed?(Choose all that apply) Why is this important?
    Answer this question
    
The signs of pulmonary hypertension include a loud pulmonary component of the second heart sound, an early systolic ejection click ora mid-systolic ejection murmur. In advanced disease, distended jugular veins with accentuated V waves on the electrocardiogram, a precordial lift from right ventricular hypertrophy, a right ventricular S4 gallop may be audible, a holo systolic murmur may be heard when tricuspid valve regurgitation is present, and diastolic murmurs may indicate pulmonary valve regurgitation. A Right ventricular S3 gallop may be audible in patients with more advanced disease.

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  • Was a medical history obtained? Why is this important?
    Answer this question
    
A complete medical history and carefully focused physical examination serves as the core of the diagnostic process. The information obtained guides the further direction of the patient’s examination, enables the clinician to make educated evaluations, and be better equipped to advise the patient. 

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  • Which of the following risk factors were elicited from the patient? (Choose all that apply) Why is this important?
    Answer this question
    
A risk factor is a variable that has a causal association with a disease or disease process; the presence of the variable in an individual or a population is associated with an increased risk of the presence or future development of the disease. Thus, risk factors may be useful for identifying subjects at increased risk for a disease or for a particular outcome that results from a disease process.

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  • Was a physical examination performed? Why is this important?
    Answer this question
    
A complete and carefully focused physical examination serves as the core of the diagnostic process. The information obtained guides the further direction of the patient’s examination, enables the clinician to make educated evaluations, and be better equipped to advise the patient. 

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  • To which of the following specialists was the patient referred?(Choose all that apply) Why is this important?
    Answer this question
    
Once a diagnosis has been made, and in many cases if a diagnosis is unclear, referral to the appropriate specialists needs to be made in a timely manner. To deliver care requires a coordinated approach that allows sharing of skills and expertise. The skills of members from all professional disciplines should be utilized. 

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  • Was a cardiovascular evaluation performed? Why is this important?
    Answer this question
    
When the diagnosis of pulmonary hypertension is suspected, a thorough initial cardiovascular evaluation is warranted to establish the diagnosis and to exclude structural cardiac lesions, as well as to characterize the patient's hemodynamics and potential response to drug therapy.

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  • Under which of the following classes of pulmonary hypertension was the patient classified? Why is this important?
    Answer this question
    
Based on the clinical history, the patient with pulmonary hypertension is assigned to a functional class (I to IV) according to World Health Organization Guidelines, which are a modification of the New York Heart Association heart failure classification. This classification scale offers aprognosis of long-term outcomes and provides treatment recommendations.

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  • Which of the following measures were not initiated to prevent/treat medication-induced pulmonary hypertension? (Choose all that apply) Why is this important?
    Answer this question
    
Patients with pulmonary hypertension should avoid anorexigens, which have been proven to exacerbate pulmonary hypertension. They need to avoid decongestant medications as they can cause vasoconstriction. Barbiturates and other drugs that depress cardiac output need to be avoided as well.

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  • Which of the following diagnostic laboratory investigations were performed?(Choose all that apply) Why is this important?
    Answer this question
    
Routine laboratory studies can provide important clues to diagnosis.

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  • Was a physical examination performed? Why is this important?
    Answer this question
    
A complete and carefully focused physical examination serves as the core of the diagnostic process. The information obtained guides the further direction of the patient’s examination, enables the clinician to make educated evaluations, and be better equipped to advise the patient. 

    Click here for additional information at ClinicalKey.com 



  • Which of the following diagnostic studies were performed?(Choose all that apply) Why is this important?
    Answer this question
    
Chest radiograph should be obtained for all patients with suspected pulmonary hypertension. The chest radiograph may show cardiac enlargement with a prominent main pulmonary artery segment. This finding requires further investigation. The echocardiographic evaluation is essential to evaluate cardiac anatomy and to rule out contributing causes. 

    (2001) 12 EPPEDC 3 279-288 



  • Was a cardiac catheterization performed? Why is this important?
    Answer this question
    
Right heart catheterization is required for all children who are undergoing an evaluation of pulmonary hypertension. Hemodynamic values obtained at catheterization can be used to predict survival.

    (2001) 12 EPPEDC 3 279-288 



  • Were measures taken to prevent pulmonary hypertensive crisis during cardiac catheterization? Why is this important?
    Answer this question
    
During cardiac catheterization, angiography is performed after hemodynamic assessment, with great care being taken to avoid precipitating a pulmonary hypertensive crisis. 

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  • Was the cause of pulmonary hypertension identified? Why is this important?
    Answer this question
    
The therapeutic approach to the child with pulmonary hypertension begins with an identification of theunderlying causes. 

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  • Was an informed consent obtained? Why is this important?
    Answer this question
    
Routine follow-up evaluations are necessary to offset any complications that may arise. 

    (2011) 23 EOMSCA 3 475-484 



  • Were the underlying causes of pulmonary hypertension treated? Why is this important?
    Answer this question
    
Measures to remove or treat the underlying cause of pulmonary hypertension include the following: • Timely corrective surgery for congenital defects • Tonsillectomy and adenoidectomy when the cause of pulmonary hypertension is the upper airway obstruction • Treatment of underlying disease, such as cystic fibrosis, asthma, pneumonia, obstructive sleep apnea, or bronchopulmonary dysplasia.

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  • Was acute vasodilator testing performed? Why is this important?
    Answer this question
    
The initial evaluation for treatment of pulmonary hypertension is assessment of response to acute vasodilator therapy with inhaled Nitrous Oxide (NO), intravenous prostacyclin, or other short-acting vasodilator. 

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  • What was the child’s response to vasodilator testing? Why is this important?
    Answer this question
    
The main determinant of treatment of chronic pulmonary hypertension is the response to vasodilator testing at cardiac catheterization. The pulmonary vasodilators are used in “responders”. For non-responders, vasodilators have limited success. Vasodilators should not be used without testing first in the catheterization laboratory.

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