OVERVIEW
Pulmonary capillary hemangioma (PCH) is an extremely rare vascular proliferative disease with a poor prognosis, and is one of the causes of pulmonary hypertension. Pulmonary hypertension is different from pulmonary arterial hypertension in terms of concept, etiology and clinical manifestations, and is not to be equated with pulmonary hypertension.
Causes
Since pulmonary capillaries are extremely rare, the etiology is still unclear, the possible causes are:
1. Hereditary causes
Capillary hemangiomas are mostly congenital, usually present at birth, and according to research findings, they may be related to their genetic factors.
2.Nutritional factors
Fetal malnutrition in the mother’s body or after birth may cause the development of blood vessels to be mutilated or malformed, resulting in hypertrophy or blockage of blood vessels and accumulation, and finally forming capillary hemangioma.
3.Accidental injury
During pregnancy or delivery, the fetus or baby suffered from shock or mechanical injury is also one of the causes of capillary hemangioma. Due to the accidental injury, the normal tissues of the mother may be affected, and the damaged blood vessels of the baby will be gradually deformed or blocked, and eventually form hemangioma.
4. Improper maintenance during pregnancy
If the mother is stimulated by chemicals, medicines and radiation during pregnancy, it may also lead to capillary hemangioma after the baby is born.
Symptoms
The most common first symptom is shortness of breath after activity, which gets worse as the disease progresses. Other common symptoms include cough, hemoptysis, fatigue, and chest pain.
Examination
1. Cardiac auscultation
Pathologic murmurs can be heard.
2. Chest X-ray
Signs of pulmonary edema.
3. Chest CT, chest angiography, magnetic resonance, etc.
For differential diagnosis.
4. Bronchoalveolar lavage
Contains a large number of ferritin-containing cells.
5. Histopathologic examination
It is the gold standard for the diagnosis of this disease. Open lung biopsy or transthoracoscopic lung biopsy is required.
Diagnosis
Depending on the severity of the disease, the examination may reveal cyanosis, jugular vein raging, P2 hyperactivity on cardiac auscultation, systolic murmur in the tricuspid valve area, hepatomegaly, and lower limb edema.
Clinically, for the existence of pulmonary hypertension, chest imaging suggestive of pulmonary edema signs, bronchoalveolar lavage fluid examination found a large number of ferritin-containing cells, the application of dilation of the pulmonary arteries of the drug treatment after the emergence of pulmonary edema signs have a certain suggestive diagnostic value, if possible, if possible to carry out diagnostic tests. Meanwhile, chest CT, chest angiography, magnetic resonance imaging and other tests are also helpful for differential diagnosis.
Histopathologic examination is the gold standard for diagnosis, and open lung biopsy or trans-thoracoscopic lung biopsy should be performed; tissue specimens obtained from puncture lung biopsy are not sufficient for diagnosis.
Treatment
Pulmonary venous hemangioma is currently aimed at relieving patients’ clinical symptoms and improving their quality of life. The treatment is based on different etiologic factors and different clinical manifestations. The general treatment options include conservative treatment and surgery.
1. Conservative treatment
Mainly supportive and complication-oriented treatment. The treatment of right heart failure includes oxygen therapy, application of diuretics, etc. Small-dose digitalis drugs can also be applied, which mainly relieves the symptoms and reduces the cardiac load, but the prognosis of the patients has not been significantly improved, and anticoagulant therapy should be cautious so as not to aggravate alveolar hemorrhage.
2. Surgery
The exact and effective treatment for this disease is lung transplantation or combined heart-lung transplantation, which is generally recommended to be carried out as early as possible, but there are cases of recurrence several months after lung transplantation.
Prognosis
The prognosis for this disease is poor, with lung transplantation required for the underlying treatment, and patients often die months after diagnosis.