Severe Pulmonary Hypertension - Nursing Case Study

Pathophysiology

• Primary mechanism: Pulmonary Vascular Remodeling - In severe pulmonary hypertension, there is an abnormal proliferation of cells in the pulmonary arteries. This results in thickening and narrowing of the vessels, increasing resistance to blood flow and causing high pressure in these arteries.

• Secondary mechanism: Endothelial Dysfunction - The inner layer of the arteries (endothelium) gets impaired, leading to an imbalance between vasodilators (like nitric oxide) and vasoconstrictors (like endothelin-1). This further constricts the pulmonary arteries, worsening the hypertension.

• Key complication: Right Ventricular Failure - The right side of the heart works harder to pump blood through narrowed pulmonary arteries, leading to right ventricular hypertrophy. Over time, this strain can cause the right ventricle to fail, a condition known as cor pulmonale. This reduces the heart’s ability to pump blood effectively, leading to symptoms like shortness of breath and fluid retention.

Patient Profile

Demographics:

52-year-old female, works as a high school teacher.

History:

• Key past medical history: Diagnosed with Pulmonary Hypertension 3 years ago, previous smoker with a history of chronic bronchitis.

• Current medications: Tadalafil, Furosemide, Warfarin.

• Allergies: Penicillin, Sulfa drugs.

Current Presentation:

• Chief complaint: Increasing shortness of breath, swelling in ankles and legs, fainting spells.

• Key symptoms: Severe fatigue, chest discomfort and palpitations, light-headedness, decreased appetite, and dry cough.

• Vital signs: Blood pressure 140/90 mmHg, heart rate 110 bpm, respiratory rate 25 breaths per minute, SpO2 88% on room air.

Section 1

New Diagnostic Results:

A series of diagnostic tests were ordered to monitor the patient's condition. An echocardiogram was performed revealing right ventricular hypertrophy and dilatation, indicative of the chronic strain on the heart due to the increased pulmonary arterial pressure. The estimated pulmonary artery systolic pressure was found to be 70 mmHg, significantly higher than the normal range of 15-30 mmHg. The Pulmonary Function Test (PFT) demonstrated a reduced Diffusion Capacity of the Lung for Carbon Monoxide (DLCO) of 60%, suggesting damage to the lung tissues and blood vessels. A chest X-ray showed signs of enlarged pulmonary arteries and right ventricle.

Change in Patient Status:

Over the next few days, the patient's condition began to deteriorate. Her respiratory rate increased to 30 breaths per minute and SpO2 dropped to 82% on room air, indicating worsening hypoxemia. Her fatigue intensified and she reported increased chest discomfort. Swelling in her ankles and legs progressed, suggesting worsening fluid retention due to right heart failure. She also began experiencing episodes of syncope, likely due to decreased cardiac output and inadequate cerebral perfusion. This change in status is alarming and indicative of decompensated right heart failure secondary to severe pulmonary hypertension. Immediate intervention is required to prevent further deterioration.

Section 2

New Diagnostic Results:

As the patient's condition worsened, additional diagnostic tests were ordered. An arterial blood gas (ABG) analysis revealed a PaO2 of 55 mmHg and a PaCO2 of 50 mmHg, indicating severe hypoxemia and hypercapnia. Her serum B-type natriuretic peptide (BNP) level was markedly elevated at 900 pg/mL, suggesting significant cardiac strain. The complete blood count showed a raised hematocrit level of 56%, indicative of polycythemia, a potential compensatory mechanism in chronic hypoxia. An electrocardiogram (ECG) displayed signs of right atrial enlargement and right bundle branch block, consistent with right heart strain.

Response to Interventions:

The patient was immediately placed on high-flow nasal cannula oxygen therapy, raising her SpO2 to 92%. Despite this, her dyspnea and chest discomfort persisted. Diuretic therapy was initiated to manage her peripheral edema, which resulted in some reduction in swelling. However, her episodes of syncope continued, and she reported increasing fatigue. Given the severity and progression of her condition, the healthcare team decided to escalate care. The patient was scheduled for a right heart catheterization to assess the severity of the pulmonary hypertension and formulate a more targeted treatment plan. This intervention is crucial in managing the patient's condition and preventing further deterioration.

Section 3

New Complications:

Over the next several days, the patient's condition further deteriorated. She developed a rapid, irregular heart rate of 120 beats per minute, identified as atrial fibrillation on her telemetry monitor, a common complication in patients with severe pulmonary hypertension due to increased atrial pressure and enlargement. Her respiratory rate also increased to 28 breaths per minute with shallow respirations, further exacerbating her hypoxemia. A repeat ABG showed a PaO2 of 52 mmHg and a PaCO2 of 55 mmHg despite the high-flow oxygen therapy, indicating worsening respiratory failure.

Response to Interventions:

Given the new onset of atrial fibrillation and worsening respiratory status, the healthcare team decided to start her on an anti-arrhythmic medication, amiodarone, and to increase her oxygen flow rate. Despite these interventions, the patient's atrial fibrillation persisted, and her SpO2 only marginally improved to 93%. The team also started her on anticoagulation therapy with heparin to prevent thromboembolic complications, which are common in patients with atrial fibrillation. The team is now considering more aggressive interventions such as non-invasive ventilation or even mechanical ventilation, given the severity of her condition and poor response to initial interventions.

Section 4

New Complications:

As the patient's clinical condition continued to deteriorate, she began to exhibit signs of right heart failure - a common complication in severe pulmonary hypertension. She demonstrated jugular venous distention and peripheral edema, which are indicative of fluid overload. Her liver span also increased, suggesting hepatic congestion. The patient further complained of increasing shortness of breath despite the escalated oxygen therapy. In addition, her urine output decreased significantly to less than 30 ml/hr, raising concerns about renal perfusion and function.

Response to Interventions:

To manage the emerging signs of right heart failure, the healthcare team initiated diuretic therapy with furosemide to help eliminate excess fluid and reduce the load on her heart. Despite this, her peripheral edema and jugular venous distention persisted. A transthoracic echocardiogram was ordered, which revealed right ventricular hypertrophy and dilation, confirming the diagnosis of right heart failure. Meanwhile, her renal function tests showed a rising creatinine level of 2.5 mg/dl and a reduced GFR, indicating acute kidney injury. The team increased her fluid intake and considered starting her on renal dose dopamine to improve renal perfusion. Given the escalating complexity of her condition, a critical care consult was also requested.

Section 5

Change in Patient Status:

Over the next 24 hours, the patient's condition took a turn for the worse. She became increasingly tachycardic with a heart rate of 130 bpm and her blood pressure dropped to 90/60 mmHg. Her respiratory rate elevated to 28 breaths per minute and she was utilizing accessory muscles to breathe, indicating increased work of breathing. On auscultation, crackles were heard in the lower lobes of her lungs. Additionally, her oxygen saturation dropped to 88% on 4 liters of oxygen via nasal cannula, suggesting worsening hypoxia. Her urine output remained less than 30 ml/hr, and her mental status began to decline with her becoming more lethargic and confused.

New Complications:

In the face of her deteriorating condition, the patient developed a new complication of acute respiratory distress syndrome (ARDS). Her arterial blood gas (ABG) results showed a pH of 7.28, PaCO2 of 50 mmHg, PaO2 of 55 mmHg, and bicarbonate level of 22 mEq/L, indicative of respiratory acidosis. Her chest X-ray demonstrated bilateral infiltrates. The healthcare team immediately increased her oxygen therapy to a non-rebreather mask and prepared for possible mechanical ventilation. Meanwhile, her renal function worsened with a creatinine level rising to 3.5 mg/dl, indicating the progression of acute kidney injury. Hemodialysis was discussed as a potential intervention. At this stage, the patient's prognosis seemed grim, and a meeting was arranged with the family to discuss her condition and possible end-of-life care.