CTEPH is a condition where permanent blood clots form in major vessels leading from the heart to the lungs, causing heart failure due to the inability to pump blood to the lungs.
CTEPH is a condition in which permanent blood clots form in the major vessels leading from the heart to the lungs, causing heart failure. (Creative Commons)
Falling sick is scary enough, but when it’s something as complex as heart failure, it is even scarier. And what can be the scariest thing at this time? Doctors not being able to find the cause of the heart failure.
If the English language ever came up with a degree of comparison after the superlative degree, this case would be the reason for it because after finally figuring out the cause of the heart failure, doctors had to stop the patient’s beating heart in order to save his life.
This happened to a 49-year-old from Chennai, who suffered from advanced heart failure, which further led to liver congestion, and kidney failure, requiring dialysis.
Initially, the cause of heart failure had not been diagnosed by the doctor and neither could a contrast CT be done due to ongoing dialysis for acute kidney failure.
The patient was finally admitted to Kauvery Hospital in Chennai with breathlessness at rest, swelling of both legs, and breathing issues at night.
After a thorough assessment, the Pulmonology team at Kauvery made a diagnosis of severe Chronic Thromboembolic Pulmonary Hypertension (CTEPH) and complex sleep apnoea, leading to heart failure.
CTEPH is a condition in which permanent blood clots form in the major vessels leading from the heart to the lungs, causing heart failure due to the inability to pump blood to the lungs. Medications or stents are typically ineffective, and surgery is the only option.
CTEPH. (Temple Health)
Let us explain in simple terms what CTEPH means:
A person’s lungs are like a pair of air balloons where they breathe in air to get oxygen.
In rare instances, small blood clots can get stuck in the tubes that carry blood from the heart to the lungs. These clots can block the blood from flowing properly to the lungs.
When the blood can’t flow through these tubes to the lungs, it puts extra pressure on the heart because it has to work harder to push the blood through. This can make the heart and lungs not work as well as they should.
As a result, the person might start feeling tired, have trouble breathing, and may even feel pain in the chest, which ultimately leads to heart failure.
CTEPH is a relatively rare condition and it occurs in a small percentage of people who have had an acute pulmonary embolism (sudden blockage of lung arteries by blood clots).
“It’s a bit challenging to provide precise population numbers, but when 100 individuals are admitted to the hospital with blood clots, approximately 3 to 4 of them are likely to have this specific condition,” Dr Srinivas Rajagopala, Senior Consultant, Interventional Pulmonology & Sleep Medicine, and Director of Transplant Pulmonology & Lung Failure Unit at Kauvery Hospital, told South First.
The underlying cause of CTEPH is the persistence of organised blood clots, also known as thromboemboli, in the pulmonary arteries. These clots develop as a result of acute pulmonary embolism, which is the sudden blockage of lung arteries by blood clots.
“Blood clots in the lungs are not uncommon. Roughly 1 in 10,000 people may experience them. They recover if they seek treatment promptly, as treatment often leads to clot dissolution. However, there are instances where clot dissolution doesn’t occur, particularly in individuals with larger clots or delayed diagnoses. The underlying cause can vary and early identification is crucial,” explained Dr Rajagopala.
He added, “Common symptoms include breathing difficulties and an inability to exercise. In individuals with a known clot, if the pressure doesn’t return to normal within three months, it may indicate CTEPH.”
“Sometimes, patients may not have a history of clots; they might present with high blood pressure within the pulmonary arteries. In such cases, screening for CTEPH is vital. This patient, for instance, didn’t have any previous clot history but exhibited significantly elevated lung pressure, likely due to long-standing issues with the heart,” explained Dr Rajagopala.
Because this 49-year-old patient was undergoing dialysis, doctors had to find another way to diagnose him. They finally settled on perfusion scans — a medical test that uses a radioactive substance to show how blood is flowing in different areas of the body — helping doctors detect issues like lung or heart problems.
This scan confirmed the diagnosis of CTEPH in his case, prompting a change in the treatment plan.
Ventilator/perfusion scan. (Wikimedia Commons)
To treat the CTEPH, the doctors at Kauvery Hospital first aggressively managed his heart failure, which eventually led to discontinuation of dialysis while being supported with oxygen, a home ventilator, anticoagulation medication, and heart support with a continuous milrinone pump.
“A standard diagnostic method is the ventilation/perfusion scan, though a pulmonary angiogram is the definitive test,” said Dr Rajagopala.
Subsequently, a carefully dose-monitored contrast CT and pulmonary angiogram were done with caution to avoid worsening of his kidneys. This confirmed CTEPH and a complex pulmonary endarterectomy surgery was meticulously planned, with inputs from specialists in Pulmonology, Cardiology, and Interventional Radiology.
The diagnosis of CTEPH typically involves a combination of medical history, physical examination, and various diagnostic tests like ventilation/perfusion lung scan, CT pulmonary angiography, pulmonary angiography, and additional tests, such as blood tests to check for clotting disorders or genetic factors that may increase clotting risk.
Pulmonary endarterectomy (PEA) is the special surgery done to treat lung conditions like CTEPH.
During this surgery, the patient is connected to a machine that acts like the heart and lungs during surgery. Doctors may even find the need to drop the temperature of the patient’s body to protect the other organs.
Once ready, the surgeons stop the patient’s heart briefly in order to clear out the clots from the blood vessels in the lungs. As soon as the clots are cleared, the surgeons restart the heart and warm up the body.
In the case of the patient in Chennai, he underwent the procedure in the able hands of Dr Kumud Dhital, Program Director of the Heart and Lung Transplantation unit at Kauvery Hospital, who has performed over 150 successful pulmonary endarterectomies till date.
“The procedure involves placing the patient on a heart-lung machine and cooling them to 20°C with carefully controlled cardiac arrest. Careful removal of all the chronic organised clots from deep inside the pulmonary arteries is crucial for a complete cure and prevention of postoperative complications, and this is why meticulous pre-operative planning is important,” said the hospital in a statement.
Post-surgery, the patient was monitored in the Heart-Lung ICU, where he made remarkable progress with normalisation of pulmonary pressure and heart function, leading to a quick discharge eight days after surgery.
Since returning home, the patient has remained off dialysis and has been able to stop the additional oxygen and heart support at home, as well as most of his medication. He continues to be on anticoagulation medication alone in order to prevent recurrence of blood clots.
He rejoined work four weeks after discharge and resumed all activities — it is now two months post-discharge. Despite his complex medical condition, he exhibited remarkable morale and resilience throughout his treatment journey, the hospital stated.
“Surgery stands as the sole curative treatment for this condition. Undergoing surgery allows one to bypass the need for tablets as the disease primarily involves a blood clot in the main blood vessel leading to the lungs. Taking oral medication cannot effectively resolve this issue. As time progresses, we persist in our efforts,” said Dr Rajagopala.
He added, “Alternate approaches, such as catheter-based interventions aimed at breaking the clot, have shown limited efficacy in this challenging condition. In cases where surgery is not a viable option, the primary objective becomes prolonging the patient’s life rather than achieving a cure.”
“We are delighted to see the results of the breakthrough surgical procedure, giving hope to many who could benefit from this. This further motivates us to continue to provide cutting-edge therapies and advanced techniques to ensure optimum outcomes every time. Kauvery Hospital continues to lead the way in revolutionising healthcare, ensuring a brighter, healthier future for all,” said Dr Aravindan Selvaraj, Co-Founder and Executive Director, Kauvery Hospitals, in the press statement.
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