It started off as an adventure that’s experienced by many immigrants who leave their homeland to travel to other countries, seeking to fulfill their dreams. To get away from the mundane battle for survival. To offer their families a better life and a better future, and to provide better education for their children. Sending a part of their hard-earned money home so their parents may also be helped. Sickness was the last thing on his mind. Something he did not want and could not afford. That was the one thing that could throw a spanner in the works.

Austria - hardships and hope
This is the story of one such Indian immigrant, Mr. Mohit Chawla. He came to Austria in 2001 with his beautiful wife, ready to work hard for the kind of life he wished to create. It was difficult to learn German and find work, but he had a strong will. As they say "Where there is a will, there is a way." That was his motto. With his keen desire to succeed in life, he prevailed over his fate.He acquired a good job and, lo and behold, the Holy Grail in the form of the Austrian citizenship. Destiny was gracious to the Chawla family and they thrived . Their perseverance was paying off.

Dark clouds
During his compulsory military training in 2006 he noticed, first, a defect in his vision and then breathlessness and fatigue during exercise.


Austrian Alps – an approaching storm behind the rare Edelweiss flower.

The military doctors diagnosed aortic valve stenosis and recommended a check-up at the University Hospital in Vienna.
Mr. Chawla did not go to the hospital until 2012. He was scared. Scared of the operation and of losing his job. By the time he arrived at the Clinic his symptoms were calling for immediate action. He had no choice.

What we found
A transthoracic echocardiogram was performed straightaway. Look below at Mr. Chawla’s aortic valve. Can you guess the degree of his aortic valve stenosis?


Echo 1. Parasternal long-axis view. The aortic cusps are thickened and do not separate during systole.

Surprisingly, there was very little calcification. Does this rule out severe stenosis? At least not in the congenital form of aortic stenosis. In such cases color Doppler may point to the presence of stenosis.


Echo 2. Parasternal long-axis view with color Doppler. Systolic turbulences are present at the valve.


While color Doppler helps to detect the presence of stenosis, it does not allow us to determine its severity. To do this we need continuous-wave Doppler, which enables us to measure flow velocities across the valve. The view below shows the maximal aortic jet velocity signal we obtained. Do you know the position it was obtained from?

The pencil probe


Echo 3. Right parasternal view. The peak aortic jet velocity was above
4 m/sec and the mean pressure gradient was more than 40 mmHg. 

We asked Mr. Chawla to lie on his right side (right lateral decubitus position), putting his right arm under his head. We then placed a cross-shaped probe known as a pencil on the right side of his sternum, approximately on the second intercostal space. This probe is ideal for measuring eccentric jets directed to the right from the narrow intercostal spaces. It does take some experience to obtain good signals because the probe does not have 2D imaging capabilities to guide its correct position.

Image 4. Pencil probe.
Using the continuity equation, we also assessed the aortic valve area, which was 0.7 cm2. Mr. Chawla had a severe aortic stenosis. This was now crystal clear.

More to learn..
There is more to learn from Mr. Chawla’s echo. Look more closely at the morphology of the aortic valve.

Echo 5. Parasternal short-axis view. Zoom view on the aortic cusps.
How many cusps do you see? Remember, always observe the opening motion of the valve when you are searching for congenital forms of aortic stenosis. Well, we had the impression that the valve was bicuspid.
Next step: Surgery - a Ross procedure
A Ross procedure was performed on 20 September 2012. His wife insisted that I should be present during the operation and inform her of its progress. As promised, I gave her an update every 40 minutes to let her know that everything was fine.
To refresh your memory: the Ross procedure is very useful in cardiac surgery. A diseased aortic valve is replaced with the person's own pulmonary valve. A pulmonary homograft is then used to replace the patient’s pulmonary valve.

Ross procedure: The aortic valve is replaced by the pulmonary
valve; the coronaries are reimplanted and a homograft
is used in place of the pulmonary valve.


Preparation of the homograft – (Cyrolife size 25mm).
No anticoagulation is required as in mechanical valve replacement. Isn’t that cool? Since Mr. Chawla was young and quite fit, he was an ideal candidate for the Ross procedure.
Surprise, surprise
The aortic valve was, indeed, thickened and stenotic. However, to our surprise the valve was not bicuspid but unicuspid.

Unicuspid valve.

The bicuspid aortic valve has an incidence of 0.9-1.36% in the general population. The unicuspid aortic valve has an incidence of 0.02%, which makes it about 50 times less common than the bicuspid valve. Unicuspid aortic valves are more common in men; the male/female ratio is 4:1.
Two sub-types of UAV have been described. One is the pinhole-shaped acommissural UAV and the second is the slit-shaped unicommissural UAV. While the pinhole-shaped UAV presents early in infancy with severe aortic stenosis, the slit-shaped UAV occurs much later, in adulthood, and has a less aggressive course. This was the morphology we found in Mr. Chawla’s case.

Happy ending
All’s well that ends well. Thankfully, the TEE showed that the autograft was not leaking. After the operation the patient was shifted to the ICU in stable hemodynamic condition. Two years after the operation the patient is doing very well. Those were his words. Happily married to his charming wife, the two of them are planning to have children. To tell you the truth, this does indeed warm my Indian heart!
When I informed him that Dr. Binder, Dr. Pistritto and I were going to write a blog post about his case he requested me to add the following sentence: "All of you - from the echocardiographers to the surgeons - are gods to me. Shukriya."
Those were his words, not mine!

Amit Vadehra and Anna Maria Pistritto.

PS: If you like our case stories tell your friends or simply forward the case story to them.

PSS: If you are interested in other cases dealing with aortic stenosis, here are some you may like to read:
North stars and aortic stenosis

Low-gradient aortic stenosis