TALES FROM THE CLINIC
-Series Editor Nidal Moukaddam, MD, PhD
In this installment of Tales From The Clinic: The Art of Psychiatry, we discuss congenital heart disease (CHD) and associated mental conditions. CHD predisposes patients to a lifetime of unique obstacles and additional comorbidities, emphasizing the importance of embedded psychiatric care from the very beginning of patients’ care journeys and across the life course.
Case Study
“Misty,” a 26-year-old woman, walks into the clinic on the day of her scheduled visit, a file pressed against her chest. When the intake clerk calls her name, she stands slowly, bracing an arm against her chair. Her walk towards the exam room attests to her health history; her posture implies chronic pain; and her slow and careful steps seem at odds with a disobedient body.
Her chart is already on the screen when she walks in. She is only in her 20s, but her history of congenital issues means that her medical record is as old as she is: congenital cardiomyopathy, ventricular hypertrophy, prolonged QT, pulmonary stenosis, and a septal defect. In the notes and margins of her medical record are more subtle descriptions, including flat labels like anxiety, depression, trauma, volatility, and noncompliance. Her visit today is not to the cardiologist, but to the psychiatrist at the cardiology clinic. Before she has spoken, a story has been written about her.
When she sits down, she glances briefly at the vital signs monitor. “I don’t know why they sent me here,” she finally says. “My heart doctor thinks I’m too emotional.” Her statement belies a deeper question: “Don't my heart issues justify my emotional issues?”
Discussion
Advances in pediatric cardiology and cardiothoracic surgery have reshaped the landscape of medicine. Once considered largely fatal in childhood, congenital heart disease (CHD) has become a chronic condition that most patients survive into adulthood. Now, nearly all children born with CHD are expected to reach adult life, creating a rapidly growing population of adults with congenital heart disease (ACHD).1,2 With this success has come a new set of clinical realities. Survival no longer marks the end of care, but rather the beginning of lifelong medical, psychological, and social complexity.
Psychiatry increasingly finds itself at the center of this evolving story. Adults with CHD carry not only repaired or palliated hearts, but also the cumulative emotional burdens of early hospitalizations, repeated procedures, uncertain prognoses, neurodevelopmental vulnerabilities, and the existential weight of living with a life-limiting illness. Psychiatric issues like depression, anxiety, posttraumatic stress disorder (PTSD), and maladaptive coping are natural consequences of the unique challenges patients with CHD face throughout their life; psychiatric and psychological support should be similarly embedded in patients’ care teams.
A Growing Population With Invisible Needs
Congenital heart disease is the most common birth defect worldwide, affecting approximately 2.8 newborns per 1000 live births.3 CHD encompasses a wide spectrum of conditions, ranging from mild, asymptomatic defects requiring minimal or no intervention to severe, complex malformations requiring multi-stage surgeries, lifelong management, or transplantation.
While severity is variable, most adults with CHD live with at least 1 noncardiac comorbidity; over 95% of ACHD patients experience medical, cognitive, or psychosocial complications that meaningfully affect their quality of life, morbidity, and mortality.4 Mental health conditions stand out among these comorbidities. Depression and anxiety are significantly more prevalent in ACHD populations,1,5 and these conditions are associated with worse cardiac outcomes, increased health care utilization, and higher mortality risk.6 Despite this evident need for more specialized care, many patients never receive formal psychiatric evaluation or treatment.
Developmental Scars Across the Lifespan
Key Takeaways
- Advances in cardiology mean that nearly all children born with CHD survive into adulthood, creating a large and growing population with complex, lifelong medical and psychological needs.
- Psychiatric comorbidities are both extremely common and consequential. Depression, anxiety, and PTSD are widespread in adults with CHD, and these psychiatric conditions as comorbidities are directly linked with worse cardiac outcomes, higher health care utilization, and increased mortality.
- What presents as noncompliance often has deeper explanations in complex patient populations. Cognitive deficits, misunderstood medical advice, and untreated trauma can drive behavior that providers may mislabel, underscoring the need for psychiatric perspectives in medical care teams.
- Specialized psychiatric and psychological providers integrated directly into cardiology clinics and heart centers are highly utilized resources in the programs that have adopted them.
- Interdisciplinary cardiac-psychiatric treatment helps patients engage more readily with mental healthcare, helps manage crises proactively, decreases stigma, and addresses a large gap in CHD patients’ current care trajectories; embedded mental health care improves both access and outcomes.
CHD begins influencing patients’ psychological well-being at birth.6 Infancy may involve prolonged hospitalizations, invasive procedures, painful stimuli, and repeated separations from caregivers. These experiences can disrupt normal attachment and sensory learning, leading to feeding difficulties, sleep disturbances, heightened reactivity, and developmental delays. Exposure to anesthesia at this stage can cause neurocognitive deficits that appear much later in life.
In childhood, frequent medical appointments and hospital stays interfere with school attendance, peer relationships, and play. Medical procedures present scary, painful experiences for children to navigate while separated from their families and friends. Children may struggle academically, experience social withdrawal, or develop anxiety and behavioral regulation issues. Feelings of being different may be understood before a child has the language to articulate them. Without counsel, these feelings may manifest as poor coping skills that the child will carry to subsequent life stages, exacerbating existing problems and creating new ones.
Adolescence introduces new challenges as teenagers’ desire for independence clashes with medical dependence; risk-taking behaviors typical of this age group may present as noncompliance. The transition from pediatric to adult care is a particularly vulnerable period as patients lose familiar providers and become increasingly responsible for their own care.7 Teenagers may also experience frustration due to problem-solving, memory, and learning deficits incidental to the treatment or management of their CHD.
Under constraints imposed by their health, adults with CHD are faced with unique decisions regarding education, employment, relationships, and family planning. Many patients persistently worry about premature death, physical decline, or becoming a burden to others. Depression, anxiety, and PTSD are common lifelong companions, even among those with mild defects.
Many adults with CHD also struggle with cognitive deficits. Hypoxia or poor oxygenation, early exposure to anesthesia, and acquired brain injuries (eg, stroke, SCA) may be incidental to the course or treatment of a patient’s CHD, but their consequences are lifelong8; as patients age, the downstream consequences of surgical interventions may manifest or worsen.9 Patients with ACHD are more likely to develop learning disabilities and may have deficits in verbal learning, written and oral processing speed, and memory.10 These challenges can affect educational attainment, employment, and patients’ capacity for complex medical decision-making. In terms of these patients’ engagement with clinical practice, these cognitive deficits may manifest as misunderstandings of their prognosis or their physicians’ recommendations.
Case Study, Continued
Misty has an extensive medical history: congenital cardiomyopathy leading to ventricular hypertrophy and prolonged QT, pulmonary stenosis, and a septal defect. She also has a history of seizures, mitochondrial myopathy, and multiple surgical procedures. Having been born with a severely compromised heart, Misty’s entire life unfolded in close proximity to medical care, yet mental health services remained peripheral until her 20s; Misty was finally referred to psychiatry when she was 26.
During her initial evaluation, she reported early childhood trauma and symptoms highly indicative of chronic PTSD, MDD without psychosis, GAD, panic disorder with agoraphobia, and borderline personality traits. She also reported constant vigilance around her heart rate and blood flow, fear of dying while asleep, shame around her dependence on others, and the emotional burden surrounding her inability to have children. She could not work because of the severe fatigue she was experiencing, and self-medicating with alcohol had become a maladaptive coping strategy.
Misty was referred to psychiatry because as her cardiac and social stressors became more complex, her emotional distress became more visible during cardiology visits. The cardiologist would use these visits as an opportunity to invite the on-site psychiatrist to speak with her, taking advantage of readily-available mental health resources to integrate psychiatric and cardiologic care. Despite this, Misty declined regular, specialized psychiatric follow-ups following her initial assessment. She believed her affect was justified by her health issues.
This dynamic persisted for years as cardiac psychology referrals were repeatedly ignored. Misty reengaged with mental health services 3 years after her initial psychiatric assessment only as her cardiac condition worsened. She needed an implantable device to regulate her heart rate and her cardiology team impressed upon her that her anxiety, though justified, was worsening her arrhythmia. The cause of her anxiety no longer mattered; whether pathological or justified, it was threatening her health, and she needed specialized mental health care.
Within a month of reestablishing mental health care, Misty disclosed an unplanned pregnancy. She was advised that continuing the pregnancy would pose a severe risk to her life, but believing her pregnancy to be a miracle, she wished to proceed. To protect the fetus, Misty discontinued all medications, including those she had been advised to continue by her cardiology and psychiatry teams.
Eighteen weeks into her pregnancy, Misty was emergently admitted with persistent symptoms of severe heart failure and was rapidly escalated to intensive care. The amount of pressors needed to keep her alive would ultimately harm her unborn child. Despite a consensus among her medical teams that continuing the pregnancy would likely result in both maternal and fetal death, Misty struggled with the decision to terminate.
In the interim of Misty’s deliberation, psychiatry and psychology worked closely with cardiology, maternal-fetal medicine, and critical care, providing noncoercive and compassionate support. After 4 days of laborious consideration, with input from her family and medical teams, Misty agreed to the elective termination of her pregnancy. While she was being dilated, before the procedure could be completed, she spontaneously delivered a nonviable fetus.
The trauma of this loss and the panicked hospitalization that preceded it presented a level of stress that few can manage alone; cardiac psychology and psychiatry were immediately present to mitigate it and to provide supportive care. In the following months, she required escalating doses of pressors to manage her hemodynamic instability and she was ultimately recommended for heart transplantation.
This recommendation was opposed by some of her providers, as they felt that her decision to get pregnant was an act of noncompliance that would exclude her from transplant candidacy. This situation presented another instance in which psychiatry and psychology’s presence was crucial to bring the team to a consensus. As it happened, Misty’s neurocognitive limitations led her to misunderstand her providers’ recommendations on multiple occasions; believing pregnancy to be impossible, not life-threatening, she did not consider that not using contraception was noncompliant and dangerous. Furthermore, a pregnancy that defied medical possibility, in her mind, was worth saving by any means necessary.
Misty remained at the ICU level of care until her transplant 6 months later. Cardiac psychology focused on coping, grounding, and meaning-making. Cardiac psychiatry adjusted her medications continuously to match the anxiety and decompensation associated with such a traumatic and complicated hospital course. Coordinating with the ICU team, mental health support was provided not with a single consult, but throughout the entirety of Misty’s hospitalization.
Following a successful transplant, her postoperative course was uncomplicated and she was discharged to home two weeks later. She continues to follow up with mental health services.
Embedded Mental Health Care
Misty may have been 26 when she presented to the ICU with symptoms of severe heart failure, but she was also a newborn once, being fussed over after prenatal ultrasounds showed that she would be born with CHD, withheld from her parents’ touch and kept in a stainless steel bassinet. She was 5 years old once, being told to slow down and be careful while her friends played tag during recess. She was 14 years old once, struggling to manage her time, wrestling with a learning disability she was unaware of and grappling with neurocognitive deficits. Not so long ago, she was 20, believing that pregnancy was impossible, grieving the possibility of becoming a parent.
CHD presents unique challenges at all stages of a patient’s life. Each of these challenges has its own consequences, and these consequences are often both poorly defined and gradually revealed—neurocognitive deficits, for example. Given the bewilderingly high prevalence of psychiatric comorbidities in ACHD patients and the rate at which these conditions go unrecognized,11 psychiatric care should be embedded in the CHD management playbook and consequently in hospitals, heart centers, and ICUs.
This would permit and normalize the management of challenges as they arise, not just when catastrophe strikes. Misty’s ambivalence toward psychiatric care could be explained by its absence; over the course of a lifetime, practically every specialty had been involved in her care except for psychiatry. Embedded mental health providers may help characterize psychological support as an innate part of cardiac treatment plans, reducing patients’ resistance to psychological and psychiatric support: when psychiatric services were integrated with Misty’s cardiology care, her engagement with these services and her outcomes improved.
Clinical guidelines from various cardiology associations recommend routine screenings for depression and anxiety in ACHD populations, with referral to mental health specialists as indicated.12,13 However, screening without access in unintegrated systems makes engagement difficult for patients who may already be overwhelmed with medical visits. Programs that embed dedicated providers within their heart centers demonstrate both improved access and improved outcomes.
At one heart center, the introduction of dedicated cardiac psychology and psychiatry services was met with 1392 outpatient referrals and 290 inpatient consultations across a wide age range. These dedicated providers were specially equipped to manage the procedural anxiety, medication coordination, interdisciplinary health questions, and unique existential concerns of ACHD patients.6
Transitioning From Surviving to Thriving
The treatment of patients with ACHD challenges providers to meet patients where medicine has carried them: into longer lives and more complex identities. Cognitive behavioral therapy and other psychological interventions may reduce depressive symptoms and improve coping, and pharmacotherapy is safe and effective when managed by an integrated care team12-14; though these interventions are only effective if they can actually reach patients. Embedding psychiatry in cardiac care and pursuing interdisciplinary clinical practice improves patients’ access to providers and normalizes psychiatric support as an integral part of CHD and ACHD management.
Ms Hashmi is a research coordinator at the Menninger Department of Psychiatry and Behavioral Sciences at Baylor College of Medicine with an interest in the overlaps between internal medicine and psychiatry.
Dr Shahin is psychiatry faculty at Baylor College of Medicine,and Texas Children’s Hospital. with a special interest on adult congential heart disease.
References
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