Physicians are familiar with the clinical sign of hemoptysis, the coughing up of blood. It is a condition that often prompts alarm in patients, but cases of hemoptysis are a common sight in the emergency department. 

The investigatory steps following the initial presentation of hemoptysis are crucial, with little margin for error. The differential diagnoses for hemoptysis are wide, ranging from the benign to the malignant. 

When evaluating cases of hemoptysis, it is important to first identify whether the patient is experiencing a “true” case of hemoptysis, meaning that the source of bleeding is from the airways or lungs. Sometimes, hemoptysis can arise from pathology in the gastrointestinal tract instead. Identifying the systems involved will help nudge physicians in the right exploratory direction. 


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The next thing to differentiate in cases of hemoptysis is whether the sputum is merely blood-tinged or whether massive bleeding has occurred. If it has, the risk of mortality jumps drastically. In Continuing Medical Education, Ittrich et al wrote, “Because of the low volume of the tracheobronchial space (150 to 200 mL), a collection of blood can swiftly cause a serious problem with gas exchange.”

All cases of hemoptysis require the close monitoring of vital signs and laboratory test results. The most crucial element of care is to ensure that gas exchange continues. Medical imaging can be conducted to pinpoint the exact area of damage, and appropriate, life-saving intervention should be initiated immediately.

Hemoptysis in the Context of PAH

Hemoptysis is one of many possible clinical manifestations of pulmonary arterial hypertension (PAH). The typical progressive changes associated with PAH are the remodeling of the pulmonary vascular bed, vasoconstriction, thrombosis, pulmonary vascular changes, and endothelial damage. These changes predispose patients to developing hemoptysis, especially when congenital heart disease (CHD) is also present. 

Read more about PAH etiology 

In the Journal of Clinical Medicine, Baroutidou and colleagues wrote about the presentation of hemoptysis in patients with PAH. They say the above physiological changes in PAH lead to 7 crucial pathologies: 

  • The erosion of hypertrophied bronchial arteries
  • The erosion of pulmonary arteries, which increases the risk of dissection or aneurysm
  • The angiomatoid lesions or the dilation of the pulmonary arteriolar wall
  • Bronchial and nonbronchial system collaterals
  • Hemostatic imbalances
  • The separation of anastomoses between pulmonary and systemic capillaries
  • Laminated thrombi in aneurysmal and calcified pulmonary arteries. 

All of these changes, individually and together, increase the risk of the patient developing hemoptysis. Let’s take the sixth point as an example—the separation of anastomoses between pulmonary and systemic capillaries. The pulmonary arteries and bronchial arteries function in sync with each other; the pulmonary arteries account for nearly all of the arterial blood supply to the lungs, while the bronchial arteries nourish the supporting airway structures. 

At the level of the vasa vasorum, these 2 systems are connected by thin-walled anastomoses. In the setting of PAH-CHD, “these fragile vessels are subjected to increased systemic arterial pressure and can lead to hemoptysis by rupturing into the alveoli or bronchial airways,” wrote Baroutidou et al. In some cases, massive hemoptysis can occur, which can be life-threatening. 

Prognosis Unclear for Patients With PAH-CHD and Hemoptysis 

Surgeons have at their disposal a number of options to treat hemoptysis. Ittrich and colleagues wrote, “Whenever possible, surgery should be an elective procedure after multidisciplinary hemostatic treatment, identification of the cause of bleeding, and definition of the necessary extent of resection.” 

Any course of action taken by the treating physicians is intended to put the patient out of immediate danger by controlling the bleeding. The case for the use of anticoagulants in this setting remains controversial. 

The prognosis of a PAH-CHD patient who presents with hemoptysis is unclear. We do not have enough long-term data to make conclusions about the impact of hemoptysis on the quality of life and the survival rates of patients with PAH-CHD. 

“The current literature establishes no impact on survival; hence hemoptysis should not be considered as a fatal clinical manifestation or a deteriorating prognostic factor in this population,” Baroutidou and colleagues wrote. 

Fatality has been decreasing in patients with PAH-CHD who present with hemoptysis. This is likely due to our improved understanding of PAH-CHD, as well as having better protocols to deal with hemoptysis in the acute setting. 

Read more about PAH treatment

Another factor that almost certainly has an impact on prognosis in these patients is whether hemoptysis recurs. Some scientists believe that the recurrence of hemoptysis in patients with PAH-CHD is a clear sign of disease progression. This should, in turn, prompt physicians to escalate PAH treatment and possibly push for a transplantation assessment. Generally speaking, the adequate removal of the source of bleeding via surgical resection reduces the recurrence rate to only 2.2% to 3.4%. 

Due to the high risk of morbidity and mortality associated with hemoptysis in the context of PAH-CHD, physicians should be adequately prepared to administer emergency treatment should the worst occur. The best way to manage hemoptysis is to do so with a “multidisciplinary, personalized decision-making approach in specialized centers,” Baroutidou and colleagues wrote.

References

Baroutidou A, Arvanitaki A, Hatzidakis A, et al. Haemoptysis in pulmonary arterial hypertension associated with congenital heart disease: insights on pathophysiology, diagnosis and management. J Clin Med. 2022;11(3):633. doi:10.3390/jcm11030633

Ittrich H, Bockhorn M, Klose H, Simon M. The diagnosis and treatment of hemoptysis. Dtsch Arztebl Int. 2017;114(21):371-381. doi:10.3238/arztebl.2017.0371