Advanced Heart Failure Therapies for Adults With Congenital Heart Disease: JACC State-of-the-Art Review
Stopping The Bias Snowball, Before It Kills Your Patient
I arrived on scene perhaps ten minutes behind the first-in crew and the shift supervisor. Both the crew paramedic and the supervisor were knowledgeable, experienced providers. A school nurse was there as well, providing background info on our patient; a 16-year-old student with a history of asthma in severe distress.
Our patient was in bad shape. She had severe difficulty breathing, tachycardia, was pale and diaphoretic, and you could hear the wheezes and rhonchi from 10 feet away. Her eyes had that unfocused, half-lidded stare that signals impending loss of consciousness and respiratory arrest.
No doubt about it, she was sick and getting worse. She hadn't responded to multiple doses of her Combivent inhaler, and the lead medic – one our our flight paramedics who was covering a shift on a ground unit that day – already had a DuoNeb in place on a non-rebreather mask he converted to a nebulizer mask. Despite that, the pulse oximeter only picked up a waveform intermittently, and when it did, her oxygen saturation was only in the 80s. The EMS crew was about to do what you'd expect anyone to do in this case – administer IM epinephrine.
Only they hadn't noticed what I had; the patient's heart rate was 268.
You'd expect a severely hypoxic patient to be tachycardic, sure, but compensatory tachycardias don't get that high. Even supraventricular tachycardia doesn't usually get that high in an adult. One of the few mechanisms that can cause a tachycardia higher than 250 in an adult is one involving an abnormal accessory pathway, like a Kent bundle.
I pointed out the heart rate to the supervisor and the lead medic, urged them to pause and assess for just a moment, and asked the school nurse for the girl's medical file.
Sure enough, there it was: Wolff-Parkinson-White Syndrome.
One synchronized cardioversion, and about 5 minutes later, the girl looked like a different patient. She was perfusing well, had a heart rate of 92, and an oxygen saturation of 100%.
WPW was right there in the girl's history, but as she took no meds for it and her initial complaint to the school nurse was difficulty breathing, the nurse got a bad case of tunnel vision and only considered status asthmaticus. Moreover, she passed that incorrect diagnosis on to the responding EMS crew, and they based their assessment and treatment on faulty information.
Now, I don't know what would have happened if we gave this young lady epinephrine, but cardiac arrest is high up on the list of possibilities.
Bias cascadeWhat happened here was a bias cascade that nearly killed a patient. First, the nurse succumbed to confirmation bias, a form of tunnel vision. She knew asthma in the patient's history, heard "I can't breathe," and stopped assessing at that point. From that point forward, the circumstances controlled her, rather than her controlling the circumstances.
Difficulty breathing? Well, let's see here … yep, she has a history of asthma.
Wheezing? Check.
Palpitations and chest tightness? Check.
Tachycardia? Check.
Albuterol doesn't help? Must be status asthmaticus. She needs IM epi and maybe magnesium sulfate, and I don't have that. Better call EMS.
The responding EMS crew fell victim to the bandwagon effect and the bias snowball, where each piece of incorrect information influences the next decision, sometimes leading to disastrous results. What stopped the snowball was a dispassionate observer who was not caught up in the scene to objectively assess the situation and realize, "One of these things is not like the other."
When we objectively examined the anomalous finding – an excessive tachycardia – then the entire faulty logic chain began to disintegrate. The lesson in that is, no matter how good or experienced you are, you should always play, "What if I'm wrong?" with your diagnosis and treatment decisions.
We teach this from the beginning of EMT and paramedic school; to form a list of differential diagnoses, obtain more data to narrow down that list to the most likely diagnosis, then provide treatment based upon that diagnosis. In a perverse way, experience as a provider works against us here; once you have become a master of pattern recognition, you are at increased risk of ignoring or rationalizing findings that don't fit the pattern.
Most protocols are set up so that the most generic treatments, the ones that may treat several pathologies and have the least potential for harm – for example, supplemental oxygen – are usually standing orders. Those with a higher risk/reward ratio and more nuanced thinking often require two brains to determine the appropriate treatment pathway, like consulting that expensively educated brain on the other end of the medical control line. Those usually fall below that line on your protocols marked in bold type: By physician order only.
The dangers of tunnel visionI happened to be the objective observer in this case, but I am as susceptible to tunnel vision and confirmation bias as anyone. Once, early in my career, I temporarily killed a patient because I discounted the possibility of encountering a profoundly hypothermic patient in 100-degree ambient temperatures of a Louisiana July. I gave him two liters of room-temperature IV fluid, because I was certain that cold, unconscious, hypotensive and bradycardic, plus a small puddle of coffee-ground emesis, equaled a patient in decompensating shock from a GI bleed.
I didn't realize my mistake until a rough transfer from my stretcher to the ED bed put the patient into VF. Luckily, the ED physician managed to resuscitate him with no deficits. That didn't stop him, however, from stepping out of the resuscitation room every 10 minutes, fixing me with a withering glare, and muttering, "80 friggin' degrees … "
It was not my finest moment as a paramedic, but I learned a valuable lesson that a cocky 25-year-old Kelly Grayson desperately needed: you are not infallible.
Avoid bias-related mistakesThere are some strategies you can employ to avoid making such mistakes with your patients. First and foremost, cultivate a relationship of trust and respect with your partner, and do not be afraid to bounce ideas off them, even if you're a paramedic and they're an EMT. It's a cliché to say, "Paramedics save lives, and EMTs save paramedics," but sayings become clichés because they are often correct.
The tool you both can use to check and recheck your biases is a decision-making model developed by Air Force Colonel John Boyd called the OODA Loop. Originally conceived as a decision-making process for fighter pilots engaging in dogfights, the OODA Loop focuses on rapid acquisition of information and acting upon it – what many of us in EMS call a "doorway assessment." The key is that you constantly re-evaluate your decision-making paradigm as the circumstances change.
Keep using that model as the call progresses and you perform your ongoing assessment, but the key is to dispassionately approach each step. Pretend it's a new patient and a new call, and see if that alters your clinical decision making.
With a good partner and application of the OODA Loop, you'll be that much better equipped to make sound treatment decisions.
5-month-old In UK Suffers Cardiac Arrest: How Common Is This Heart Ailment In Children?
Heart health is one of the most discussed topics in the world of medicine. In 2019, coronary heart disease was known as the world's single biggest killer. However, when one discusses heart diseases, we most often refer to men or women being victims of it.
However, a recent incident has thrown the spotlight of heart diseases, namely cardiac arrests, in small children, even as young as just few months old. Last Thursday (2 May), a five-month-old boy suffered a cardiac arrest at Legoland Windsor Resort in the United Kingdom. While the baby is in critical condition in hospital, a 27-year-old woman has been arrested on suspicion of neglecting a child to cause unnecessary injury.
We take a closer look at how prominent is the problem of cardiac arrests in children and the reasons behind it.
Cardiac arrest in the young
On 2 May, a five-month-old boy suffered a cardiac arrest while he was at Legoland Windsor in the UK. According to authorities, he is still in a critical condition in hospital.
Detective Con Zoe Eele, of Thames Valley Police's Child Abuse Investigation Unit, was quoted as telling the BBC: "Firstly, our thoughts are with the family of the boy who is in a critical condition in hospital after suffering a cardiac arrest.
"We are working closely with the team at Legoland Windsor Resort but would like to speak to anyone who have information about this incident, specifically anyone who was queueing for the Coastguard HQ boat ride between around 11.30am and 12:45pm. I would ask for the public to please avoid speculation about the incident and to respect the boy's family at this deeply upsetting time."
And the case of the five-month-old isn't a single incident. Earlier in January, a five-year-old girl died in Uttar Pradesh's Amroha of heart failure while watching cartoons on her mother's phone. Her medical reports later confirmed that her death was caused due to a sudden heart attack.
Back in May last year, a 16-year-old boy from India's Telangana was out shopping when he experienced chest pain and collapsed; he was immediately taken to a local hospital. Unfortunately, he passed away.
Experts note that while sudden cardiac arrests (SCA) among children and the young are rare, it does happen. In fact, according to Children's Hospital of Philadelphia, each year, SCA claims the lives of over 2,000 children and adolescents in the US and accounts for approximately three-five per cent of all deaths in children aged 5-19 years. It is also responsible for 10-15 per cent of sudden unexpected infant deaths.
Weak hearts of the young
But what's causing young children to suffer sudden cardiac arrests ? Doctors note that in young people, sudden cardiac death is most often genetic or congenital in nature. Acquired forms of heart disease that lead to sudden cardiac death are rare but possible.
As per a report in Medical News Today, one of the most common causes of sudden cardiac arrest in young people is hypertrophic cardiomyopathy (HCM). This occurs when the walls of the heart become thicker and stiffen, which decreases the amount of blood that can flow through the heart – this structural problem can lead to dangerous arrhythmias and even cause cardiac arrest.
Irregular heart rhythms (arrhythmias) can also lead to SCA, say experts, adding these happen when the electrical signals in the heart are delayed or blocked. Some examples of paediatric arrhythmias include long QT syndrome (LQTS) and Wolff-Parkinson-White syndrome. In LQTS, which patients suffer fast and chaotic heart rhythm, whereas Wolff-Parkinson-White syndrome is when the electrical pathways in the heart malfunction.
Other coronary artery abnormalities can also cause SCA among children.
As Dr Sandeep Gore, Department of Emergency Medicine, Fortis Hospital in Mumbai, was quoted as saying that while cardiac arrests are extremely rare, children can indeed suffer from them, particularly if they have congenital or acquired heart diseases or have experienced chest trauma.
Symptoms to watch out for
Unfortunately, as the name suggest, sudden cardiac arrest in children has no warning signs. However, some of the children who have undergone this experience, say that they complained of shortness of breath, fainting and chest pain.
Sadly, people often dismiss the symptoms as minor.
Also read: Can you exercise yourself to a cardiac arrest?Prevention is better than cure
Medical experts note that with the right precautions, the risks of SCA can be negated. Doctors say it's mandatory that parents take their little ones to a paediatricians for check-ups. Ensure that full examinations and tests are taken to help identify risk factors that may contribute to SCA.
In case, the child is engaging in sports, ensure that the child undergoes a thorough physical exam. This is another opportunity for their doctor to spot heart problems.
Also, in case, a child does suffer from SCA, adults should be aware of what to do — call for help and perform high-quality cardiopulmonary resuscitation (CPR). However, because of the difference in weight and body size, the techniques for performing CPR are different when responding to infants, children, and adults.
With inputs from agencies
Xander Wehrens Lab
Content
Current major projects in the lab focus on the molecular basis of inherited arrhythmia syndromes like catecholaminergic polymorphic ventricular tachycardia (CPVT), congenital long QT syndrome (LQTS), and Wolff-Parkinson-White syndrome (WPW), as well as other more complex, multifactorial cardiac diseases like atrial fibrillation and hypertrophic cardiomyopathy. These translational studies are aimed at developing new therapeutic targets for heart disease.
Proper contraction and relaxation of the heart depends on the coordinated flux of calcium ions in and out of individual cardiac muscle cells. Research in our lab focuses on understanding how these calcium dynamics are regulated in normal and diseased hearts, and how abnormal intracellular calcium release may contribute to the development of cardiac arrhythmias, hypertrophy, and heart failure.
We are particularly interested in the genetic and posttranslational regulation of key molecules involved in intracellular calcium handling, including the ryanodine receptor type 2 (RyR2), striated muscle preferentially expressed protein kinase (SPEG), junctophilin-2 (JPH2), and nucleoside-diphosphate kinase (NDPK). In addition, we study the paracrine signaling interactions between cardiac fibroblasts and myocytes, and their role in profibrotic signaling.
New therapies in development include small molecule inhibitors of the RyR2 calcium release channel and gene therapy vectors for CRISPR/Cas9-mediated genome editing.
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