Pulmonary Vascular Disease Clinic

Major Diseases Managed in our Pulmonary Vascular Clinic

• Pulmonary embolism and Deep venous thrombosis: Pulmonary embolism refers to a disease when a blood clot in another part of the body (typically from the lower limbs due to prolonged immobilization) breaks free and is carried by the bloodstream to the lungs. The clot, or the smaller fragments it may break into, can block the flow of blood in the lungs. In our clinic, this is treated with blood thinners and the risk of recurrence and bleeding is estimated by using validated “RISK scores”. In some conditions, like APLA syndrome, blood thinners are continued life-long but typically it is for 6-12 months.
• Pulmonary vasculitis: These are rare diseases that can affect the small blood vessels within the lung where oxygen exchange happens. They can present with blood in the sputum, breathlessness and cause life-threatening bleeding into the lung
• Pulmonary hypertension: It is important to diagnose where the high blood pressure in the lungs originates to advise appropriate treatment. This can be one of the 5 sub-groups
  • Pulmonary Arterial Hypertension (PAH): This is an increase in the blood pressure in the pulmonary arteries which carry blood from the heart to the lungs. The increased pressure cannot be measured by an ordinary blood pressure cuff; it is detected by Echocardiography, which is an ultrasound examination of the heart. It can also be detected by chest radiography and electrocardiography. The suspected high pressure can ONLY be confirmed by measuring it directly using a thin catheter into the pulmonary artery, called right heart catheterization. This measurement can give clues about where the high pressure started within the lungs and helps in titrating PAH medications and gives valuable prognostic information. PAH is caused by drugs/toxins, human immunodeficiency virus infection, autoimmune diseases like Scleroderma/mixed connective tissue disease and liver diseases. Pulmonary artery hypertension can also be familial or due to sporadic mutations of one’s DNA and is called iPAH (idiopathic pulmonary arterial hypertension). Certain rare diseases called pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) need to be distinguished from iPAH as iPAH treatment can worsen these diseases. PAH is the only group of diseases for which there are several groups of approved medications (tablets, inhaled and injections).
  • Pulmonary Venous Hypertension: This is increased blood pressure in the pulmonary veins that carry blood from the lungs to the heart. It is often caused by congestive heart failure or a damaged valve in the heart. This is treated by reducing fluid within the lungs, improving heart function or fixing the affected valve within the heart.
  • Pulmonary hypertension due to advanced lung, neuromuscular and sleep disorders: Advanced lung diseases, muscle weakness and sleep disorders likely sleep apnea (OSAHS) or obesity hypoventilation syndrome (OHS) can cause low oxygen and high carbon dioxide levels and lead to pulmonary hypertension. Treatment of this condition is by oxygen supplementation and/or portable ventilator (BiPAP); PAH medications can worsen this condition and it is important to avoid them. Persisting pulmonary hypertension due to advanced/worsening lung disease indicates the need for lung transplantation
  • Chronic Thromboembolic Pulmonary hypertension: in 3-4% of patients with acute pulmonary embolism (see above), this does not resolve and leads to worsening pulmonary hypertension due to narrowed pulmonary vessels. This can also be due to unrecognized recurrent small pulmonary embolisms. This is potentially a serious disease but is also the only curable cause of pulmonary hypertension. Treatment is by a surgery called pulmonary endarterectomy (PEA); blood thinners are continued lifelong.
  • Other specific diseases: like sarcoidosis can be associated with pulmonary hypertension by affecting multiple areas. Management is complex but is associated with good outcomes with an experienced team.

The pulmonary vascular disease clinic at Kauvery is holistic and has

• A pulmonologist/sleep specialist
• A cardiologist to perform right heart catheterization and evaluate/optimize heart failure and valvular diseases
• A rheumatologist: to manage/optimize rheumatological diseases affecting the lungs
• A lung transplant physician: for end-stage lung disease and worsening pulmonary hypertension, on maximal medical treatment
• A cardiovascular surgeon: For pulmonary thrombo-endarterectomy evaluation

What is special about Kauvery Pulmonary vascular disease clinic?

1. Evidence and score-based treatment for PTE, including for recurrence and bleeding risks
2. Diagnosis and effective management of vasculitis, including rare syndromes. Our pulmonologists have several research papers on this group of conditions
3. Prostanoid Therapy for Pulmonary Arterial Hypertension: Prostanoids are drugs used to decrease blood pressure in iPAH. They are vasodilators which means they cause the opening up of the blood vessels to allow for better flow. In many cases, the continued use of prostanoids can result in a reduction of the strain on the heart and which allows it to shrink back to its normal size and function more efficiently. Prostanoids can be used by the oral, inhaled or subcutaneous route, depending on the severity of the PAH. The Kauvery Vascular Diseases Clinic is one of the few PH clinics in the country offering Prostanoid therapy, including Remodulin therapy
4. Integrated Lung Transplant Referral and Evaluation: When PAH is so advanced that medication and other treatment options are not viable, a lung transplant is often the only option. The first stage in the process is a referral. This means that a patient with PVD is referred for a specialized diagnosis and the extent of the problem. Once this is done, the evaluation process begins.
5. Integrated Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Evaluation and Pulseless Electrical Activity (PEA)

Pulmonary Vascular Diseases are major health problems and should never be taken lightly. If you have been diagnosed with PAH or suspect that you may have the condition, you should consult a specialized lung disease treatment center. The Lung Center at Kauvery Hospital has the leading specialists in the field, the most up-to-date medical technology and equipment and the best recovery care available in India. From examination, testing, diagnosis, treatment and, if required, transplant or other surgery, all that is needed to treat PVD in the best and most advanced manner possible is available here. Contact the hospital for an appointment to begin the journey to recovery.