WHAT IS LEFT PARTIAL ANOMALOUS PULMONARY VENOUS RETURN?
WHAT IS LEFT SIDE PULMONARY ANOMALOUS VENOUS RETURN?
What is left side pulmonary anomalous venous return? Pulmonary anomalous venous return is another rare condition I thought I would never now about, but now I need to know.
Left Partial Anomalous Pulmonary Venous Return is a congenital heart defect that is present at birth.
This defect is due to some of the pulmonary vein or veins incorrectly attaching to the heart’s right atrium or other blood vessels. The left side pulmonary anomalous vein should instead connect to the left atrium.
If the left side pulmonary anomalous venous return occurs then it causes some of the lungs oxygen rich blood to mix with oxygen poor blood.
The outcome of Left Side Pulmonary Anomalous Return depends on the size of the vein or veins and if the patient is symptomatic.
THIS IS OUR EXPERIENCE WITH LEFT PARTIAL ANOMALOUS PULMONARY VENOUS RETURN
JUNE 2019
My eyes dart around the Mayo Clinic’s well lit pediatric surgical waiting room and it feels surreal.
I am waiting for the cardiac surgeon team to send me a text that our daughter is off the heart and lung machine. Our daughter is having open heart surgery.
The Mayo Clinic uses texts to update loved ones in the waiting room of the patient status.
While I wait for the text update there is a numbness in my body that is fueled by fear. My swirl of emotions exceed worry, anxiousness and apprehension.
Trying to keep my numb mind at peace I glance around the Mayo Clinic Surgical waiting room and I curiously watch how people release their worry.
I observe a sixty something Indian man who is short in stature but wide in berth pace across the aqua green carpet that lines the waiting room, while a frazzle looking mid-aged woman tries to read the hardback Tami Hoag novel, The Boy.
As I watch her frantically turn the pages of The Boy it is apparent she is not reading. The book’s pages skip through the air as she anxiously uses her right index finger to twirl the bright turquoise strands that frame of her worn face.
Laying behind the book reader is a petite Muslim lady who squishes her body to contour one of the waiting room loveseats. She unrolls part of her beige hijab to cover part of her body as she naps.
My focus shifts to the lady’s for about five minutes and I sigh as I watch the book reader rise up from her chair with the novel in hand and begin to pace the waiting room.
I understand that pacing can be a stress reliever.
My husband is a pacer and so is my oldest daughter. I often tell them to stop pacing in our house.
It drives me crazy when they pace circles in our home. The anxiety that pacing relinquishes for them drains into me.
When I am in a stressful situation I tend to defend, I try to find an easy way out and then I recoil.
A new layer of stress, worry and the need to defend occurs March of 2019.
This is a story of a negligent cardiologist who could have caused our daughter’s catastrophe consequences.
It is also the story another doctor who went the additional mile and the gracious, brilliant surgeon who saved our daughter.
MARCH 2019
My mind spins, I become dizzy and then I start to become defensive as I speak to my Pulmonologist.
Our conversation goes along these lines:
Pulmonologist: I ask the Radiologist to double read your daughter’s CT with contrast.
Me: OK
Pulmonologist: Your daughter has Left Partial Anomalous Pulmonary Venous Return (PAPVR) and then he adds, the fact that her anomaly is on the left side is extremely rare.
Only about 2% of people who have Left Partial Anomalous Pulmonary Return have the congenital heart condition on the left side.
Me: Can you repeat that?
Pulmonologist: He does and repeats that my daughter’s condition is extremely rare.
Me: I ask if he has ever had a patient with Partial Anomaly Pulmonary Venous Return?
Pulmonologist: I have one patient with her condition.
Me: So, how did they manage this congenital heart condition?
Pulmonologist: I do not know, the patient is your daughter.
Me: Really, I can’t believe our rare disease home, it surreal.
Pulmonologist: You could never made up your household’s medical journey. Yes Lisa, it does seem surreal.
Me: I go on to say, “ yes this is crazy, my husband had brain surgery last year, I live with numerous chronic ailments and now my youngest daughter has a rare congenital cardiac condition. What do I do next?
Pulmonologist: This is where my medical skills leaves your daughter and you need to seek the help of your daughter’s cardiologist. He proceeds to tell me that he has already spoke to my daughter’s pediatric cardiologist and he is aware of her condition.
Me: I have to go back to a doctor who is unable to read an Echocardiogram or CT correctly? A pediatric cardiologist who never measures her oxygen saturation rates while walking?
The pediatric cardiologist that told me her resting heart rate of 110 is normal.
A Cardiologist who watches my daughter almost collapse on the treadmill test and then he arrogantly tells me that my daughter does not have a cardiac issue. She needs to see a pulmonologist.
I go on to remind my pulmonologist that he is the medical expert who had the wisdom to call the Radiologist and ask him to review the CT.
After my comment there is silence on the phone and what I perceive to be an unspoken understanding.
I then proceed to thank my Pulmonologist for his open mind, kindness and support.
I have known my Pulmonologist for over fifteen years.
He guides me through my journey with Pulmonary Embolisms, helps me to manage my Relapsing Polychondritis, always offers an open ear and he tries to navigate me in the correct direction.
After my Pulmonologist welcomes my daughter to his practice he runs numerous tests and orders lab tests.
All test results are negative. I begin to wonder if he thinks I have Munchausen Syndrome.
I insist to the doctor that my daughter is not well and he needs to run more tests.
To appease me the doctor orders a six minute test on my daughter. She fails the six minute walk, completely. The six minute walk shows that my daughter’s body does not receive enough oxygen when exercising.
The Pulmonologist questions the results and he reruns the six minute walk. Again, the well below normal results show my daughter’s body does not receive enough oxygen.
The Pulmonologist tells me that he will phone the Radiologist and ask for a review of the CT with contrast.
When the Radiologist reviews my daughter’s CT it is obvious that my daughter has Left Side Partial Pulmonary Venous Return. The oversight of my daughter’s CT could have caused her death.
After the misread of the CT my husband and me know we need more medical assistance.
MEDICAL STRUGGLES BEGIN WITH CHOC PEDIATRIC CARDIOLOGIST AHMAD ELLINI
Condescending, arrogant, close minded are words that come to mind when I reflect on pediatric cardiologist Ahmad Ellini medical behavior.
WHY I QUESTION CARDIOLOGIST ELLINI MEDICAL SKILLS?
At our first appointment January 2018, Doctor Ellini confirms to my husband and me that he did review Isabella’s Echocardiogram and there are no abnormalities.
I am insistent that it seems like our daughter has some form of cardiac condition.
I convey that our daughter, Isabella has shortness of breath whenever exercising. Dr. Ellini of CHOC of Orange agrees to a two week heart monitor hostler.
I convey that Isabella is a cheerleader and she is involved in cheer stunts. Dr. Ahmad Ellini says it is alright to remove her heart monitor during stunts.
Isabella’s heart monitor results return as non remarkable.
I insist to Doctor Ellini that Isabella’s fatigue continues to grow and everyday seems to be more of a challenge. Our daughter’s fatigue to causing her to miss school.
TREADMILL TEST APRIL 2018
Fortunately, Dr Ellini agrees to a treadmill test. My daughter struggles during the treadmill test.
She keeps gasping for air during the test, her legs become weak as she stumbles more then jogs on the treadmill. It is obvious she can not longer proceed.
In an authoritative voice Dr. Ellini tells me that Isabella does not have a cardiac issue and she needs to be seen by a pulmonologist.
As March 2018 turns to October 2018 my daughter’s fatigue, chronic pain, and headaches increase.
As 2018 turns into 2019 my concerns about my daughter’s health increase.
I constantly remind my daughter to use her asthma inhaler.
My daughter insist the inhaler is worthless, and states the medication does not provide her with any relief.
Unbeknownst to me, she is correct.
JANUARY 2018 PILATES CLASS
At the beginning of a Pilates bar class I watch my daughter slowly faint in front of me.
Once my daughter is up to walking to our car I immediately bring her to her pediatrician’s office.
The pediatrician ask if she was hot before she fainted, did she use her inhaler, did she have breakfast.
My daughter replies, no it was not hot in the Pilates class, she used her inhaler and she had eaten breakfast.
The doctor says, she is fine. My brain screams no she is not!
JANUARY 2018 EMERGENCY ROOM
Two days after my daughter faints she is lying in her bed with teary eyes. She feels dizzy and she has a headache.
I bring my daughter to the Emergency Room and I insist to the Emergency Room doctor that she needs an EchocardIogram.
The doctor runs labs on my daughter and then writes the order for an Echocardiogram.
Hours pass as we wait for a pediatric radiologist to read my daughter’s Echo.
The Emergency Room doctor tells my daughter and me that her labs and Echocardiogram are normal and he is releasing her.
I am not happy when I learn that all my daughter’s tests are unremarkable. My gut screams there is something wrong.
We later learn, yes, something is wrong. The Radiologist misread my daughter’s Echocardiogram.
REWIND TO MARCH 2018
Doctor Ahmad Ellini runs a Bubble Echo and orders a CPed test.
As Doctor Ellini performs the bubble echo, my husband and me can see the massive left side partial pulmonary return.
When I ask Dr. Ellini about the right side of her heart being enlarged he is quick to be dismissive.
Cardiologist Ahmed Ellini keeps insisting to my husband and me that our daughter’s heart anatomy is normal.
If our daughter had issues then there would be a hole or holes in her heart. He rattles that 85% of the patients with partial pulmonary venous return have a hole in the heart.
I then say to Doctor Ellini, that my daughter’s condition is rare and someone has to be the rare one on the 15% side.
I question Dr. Ellini how normal can my daughter’s heart be if an extremely large pulmonary venous return does not bring her body oxygen.
I then emphasis to Dr. Ellini that my daughter has numerous symptoms of partial pulmonary venous return.
PARTIAL ANOMALOUS PULMONARY VENOUS RETURN SYMPTOMS
- Headaches
- Fatigue
- Dizziness
- Shortness of Breath Exercising
Cardiologist Ellini discounts my daughter’s condition and conveys to my husband and me that there is no way the Partial Pulmonary Anomalous Return could cause her symptoms.
Dr Ellini goes on to say that there must be something else that is triggering her symptoms.
CARDIOPULMONARY EXERCISE CPED TESTING AKA CPED
CHOC CPED
Cardiopulmonary exercise testing includes the measurement of respiratory gas exchange and is the gold standard for determining aerobic fitness, as well as for examining the integrated physiological responses to exercise in pediatric medicine.
My husband and me arrive at CHOC of Orange County to have our daughter observed on the CPED.
One look at the the CPED bicycle and I think, wow, this bike and machines look outdated.
I have looked up CPED images and centers on the internet and the bicycle equipment did not appear the same.
Now, I can’t make this up. When a nurse is adjusting Isabella to the bike and equipment another nurse comes into our room and says, “Great News! We are getting new bikes!” Oh, yay says the other nurse. These CPED bikes are fourteen years old.
The CPED bike looks out of date. There is no mask to place on my daughter’s face just a blue plastic clip to pinch her nose shut. She must breath in and out of a hose when cycling.
The nurse states he needs to set the resistance in correlation to her body and weight. I mention to the nurse that my daughter may be thin but she is strong.
Fifteen minutes pass and the CPED test is over.
As soon as the nurse takes the hose out of my daughter’s mouth and remove the clip her nose it is apparent that she is upset.
My daughter tells me that there was no resistance on the bike. Mom, “it was like peddling in the air.”
I convey this to the nurses and doctor.
My husband and me are told by the doctor that she passed the CPED test.
I proceed to tell the doctor that my daughter felt there was no resistance.
She only has shortness of breath when she is exercising. The bike cycling in the air did not feel like exercise to my daughter.
The doctor goes on to say, that does not matter. She did great!
I know in my gut, this doctor is incorrect.
Dr. Ellini phones me to tell me that Isabella does not have a decrease in oxygen when exercising. All of her gases look good.
I inquire why did she fail numerous six minute walks at the Pulmonologist office?
Dr. Ellini goes on to say, the CPED is the gold standard and my Pulmonologist must have broken equipment.
I say, really?
I go on to say that I have placed a highly rated oxygen saturation meter on my daughter index finger while she rides on our home’s recumbent bicycle.
The results are the same, within one minute on the recumbent bike you can watch her oxygen saturation rates go below 90.
Dr. Ellini, says, “You can’t go by internet equipment.”
I ask Dr. Ellini, can you please give Isabella a six minute walk?
Dr. Ellini says, I do not offer six minute walks.
As I end my phone call with Dr. Ellini, I have no doubt I need another avenue of medical care for my daughter.
MAYO CLINIC CARDIAC SURGEON JOSEPH DEARANI
My no-copay 24/7 Doctor Google Scholar enables me to find an medical article about Partial Pulmonary Anomalous Return that is co-authored by Dr. Dearani.
Our family will be forever grateful to Mayo Clinic’s Dr. Dearani, Dr. Johnson and their medical team.
I sent my daughter’s medical records, Echocardiogram and CT to Dr. Dearani.
Within a week Dr. Dearani and Dr. Johnson provide me with write up about my daughter’s health condition.
Dr. Dearani and Dr. Johnson both confirm that the right side of our daughter’s heart is enlarged.
Fortunately, we are able to make an appointment to see Dr. Dearani.
MAY 2019 CARDIOLOGIST DOCTOR JOHNSON
Dr. Johnson patiently explains our daughter’s medical condition to us. He explains how the surgery for her Left Partial Pulmonary Anomalous Return would be accomplished and the follow-up care.
My husband and me, ask Dr. Johnson if our daughter’s cardiac surgery will be easy.
Dr. Johnson says, yes, it is an easy surgery if you have the right surgeon.
JUNE 2019 DOCTOR DEARANI
Doctor Dearani is the right surgeon for us!
Fortunately for us, Dr. Dearani is able to perform open heart surgery to repair our daughter’s Left Partial Pulmonary Anomalous Return.
I must note that Dr. Dearani is a brilliant surgeon, who is open to questions, follows up with his patients and has saved the lives of so many.
I will be forever grateful that I was able to contact the Mayo Clinic prominent cardiac surgeon and arrange open heart surgery.
Thank you, Dr. Dearani. Your medical talents will enable our daughter to have a wonderful life.
RECOVERY 2019
It is still June and it is three weeks after my daughter’s open heart surgery. With delight I blog that my daughter is making incredible strides.
She is building her cardio endurance by attending a cardiac rehab three times a week.
We are blessed that her surgery is one and done.
Doctor Dearani is able to successfully repipe / rewire my daughter’s heart.
LESSONS LEARNED FROM RARE LEFT PARTIAL PULMONARY ANOMALOUS RETURN
- Trust your gut
- Doctors, Medical Specialist eg. Radiologist can make mistakes
- Somebody has a rare condition
- Second opinions may save your life
So, that is the medical background to my daughter’s Left Partial Pulmonary Anomalous Return.
I hope that my daughter’s medical experience may help someone else.
If you are interested in viewing the pictures of my daughter’s Left Partial Pulmonary Anomalous Return open heart surgery then click on this web link.
Again, thank you Dr. Joseph Dearani and the Mayo Clinic!
Resources:
https://www.sciencedirect.com/science/article/pii/S000349750701140X
https://www.sciencedirect.com/science/article/pii/S0003497502045472
https://europepmc.org/abstract/med/2225404
https://www.atsjournals.org/doi/full/10.1513/AnnalsATS.201611-912FRp>
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