Cardiac Physiology and Pathophysiology in Pregnancy.

Cardiovascular disease is the leading indirect cause of maternal morbidity and mortality, accounting for nearly one third of maternal deaths during pregnancy. The burden of cardiovascular disease in pregnancy is increasing, as are the incidence of maternal morbidity and mortality. Normal physiologic adaptations to pregnancy, including increased cardiac output and plasma volume, may unmask cardiac conditions, exacerbate previously existing conditions or create de novo complications. It is important for care providers to understand the normal physiologic changes of pregnancy and how they may impact the care of patients with cardiovascular disease. This review outlines the physiologic adaptions during pregnancy and their pathologic implications for some of the more common cardiovascular conditions in pregnancy.

Permanent Pacemaker-Mediated Exertional Hypoxemia in a Patient With Ebstein Anomaly.

Patients with Ebstein anomaly are known to have a higher incidence of interatrial communications and shunting of blood and its components through, mainly due to either streaming of tricuspid regurgitation or due to elevated right atrial pressure. Here we describe a case where permanent pacemaker lead kept a patent foramen ovale open leading to right-to-left shunting of blood and exertional hypoxemia. This is the first such case report in the published literature.

Adult Congenital Heart Disease Care in Canada: Has Quality of Care Improved in the Last Decade?

BACKGROUND: Patients with adult congenital heart disease (ACHD) are at increased risk of comorbidity and death compared with the age-matched population. Specialized care is shown to improve survival. The purpose of this study was to analyze current measures of quality of care in Canada compared with those published by our group in 2012. METHODS: A survey focusing on structure and process measures of care quality in 2020 was sent to 15 ACHD centres registered with the Canadian Adult Congenital Heart Network. For each domain of quality, comparisons were made with those published in 2012. RESULTS: In Canada, 36,708 patients with ACHD received specialized care between 2019 and 2020. Ninety-five cardiologists were affiliated with ACHD centres. The median number of patients per ACHD clinic was 2000 (interquartile range [IQR]: 1050, 2875). Compared with the 2012 results, this represents a 68% increase in patients with ACHD but only a 19% increase in ACHD cardiologists. Compared with 2012, all procedural volumes increased with cardiac surgeries, increasing by 12% and percutaneous intervention by 22%. Wait time for nonurgent consults and interventions all exceeded national recommendations by an average of 7 months and had increased compared with 2012 by an additional 2 months. Variability in resources were noted across provincial regions. CONCLUSIONS: Over the past 10 years, ACHD care gaps have persisted, and personnel and infrastructure have not kept pace with estimates of ACHD population growth. Strategies are needed to improve and reduce disparity in ACHD care relative to training, staffing, and access to improved care for Canadians with ACHD.

The Impact of the COVID-19 Pandemic Restrictions on the Provision of Adult Congenital Heart Disease Care Across Canada: A National Survey.

Background: The COVID-19 pandemic significantly impacted health care access across Canada with the reduction in in-person evaluations. The aim of the study was to examine the effects of the COVID-19 pandemic on access to health care services among the Canadian population with adult congenital heart disease (ACHD). Methods: All Canadian adult congenital heart affiliated centres were contacted and asked to collect data on outpatient clinic and procedural volumes for the 2019 and 2020 calendar years. A survey was sent detailing questions on clinic and procedural volumes and wait times before and after pandemic restrictions. Descriptive statistics were used with the Student t-test to compare groups. Results: In 2019, there were 19,326 ACHD clinic visits across Canada and only 296 (1.5%) virtual clinic visits. However, during the first year of the pandemic, there were 20,532 clinic visits and 11,412 (56%) virtual visits (P < 0.0001). There were no differences in procedural volumes (electrophysiology, cardiac surgery, and percutaneous intervention) between 2019 and 2020. The mean estimated wait times (months) before the pandemic vs the pandemic were as follows: nonurgent consult 5.4 ± 2.6 vs 6.6 ± 4.2 (P = 0.65), ACHD surgery 6.0 ± 3.5 vs 7.0 ± 4.6 (P = 0.47), electrophysiology procedures 6.3 ± 3.3 vs 5.7 ± 3.3 (P = 0.72), and percutaneous intervention 4.6 ± 3.9 vs 4.4 ± 2.3 (P = 0.74). Conclusions: During the pandemic and restrictions of social distancing, the use of virtual clinic visits helped to maintain continuity in ACHD clinical care, with 56% of ACHD visits being virtual. The procedural volumes and wait times for consultation and percutaneous and surgical interventions were not delayed.

Contexte: La pandémie de COVID-19 a eu des répercussions sur l'accès aux soins de santé partout au Canada, y compris une diminution des évaluations en personne. La présente étude visait à évaluer l'effet de la pandémie de COVID-19 sur l'accès aux soins de santé chez les adultes atteints de cardiopathie congénitale. Méthodologie: Nous avons communiqué avec tous les centres canadiens de prise en charge de la cardiopathie congénitale chez l'adulte et nous leur avons demandé de recueillir des données sur les consultations externes et le volume des interventions pour les années 2019 et 2020. Un sondage détaillé leur a été transmis sur les volumes de consultations et d'interventions et sur les temps d'attentes avant et après la mise en place de restrictions liées à la pandémie. Les groupes ont été comparés lors d'une analyse statistique descriptive utilisant le test t de Student. Résultats: En 2019, 19 326 consultations pour cause de cardiopathie congénitale chez l'vadulte ont été enregistrées au Canada, dont seulement 296 (1,5 %) ont eu lieu en mode virtuel. Au cours de la première année de la pandémie, 20 532 consultations ont été relevées; 11 412 (56 %) ont été menées virtuellement (p < 0,0001). Aucune différence n'a été observée dans le volume d'interventions (interventions en électrophysiologie, interventions chirurgicales et interventions percutanées) entre 2019 et 2020. Les temps d'attente moyens estimés en mois, avant et pendant la pandémie, étaient les suivants : consultations non urgentes, 5,4 ± 2,6 vs 6,6 ± 4,2 (p = 0,65); interventions chirurgicales, 6,0 ± 3,5 vs 7,0 ± 4,6 (p = 0,47); interventions en électrophysiologie, 6,3 ± 3,3 vs 5,7 ± 3,3 (p = 0,72); et interventions percutanées, 4,6 ± 3,9 vs 4,4 ± 2,3 (p = 0,74). Conclusion: Au cours de la pandémie et de la période où les restrictions de distanciation sociale étaient en vigueur, le recours aux consultations virtuelles dans les cliniques a contribué à la continuité des soins offerts aux adultes atteints de cardiopathie congénitale, puisque 56 % des visites se sont déroulées virtuellement. Le volume d'interventions n'a pas été touché et les temps d'attentes pour les consultations, les interventions percutanées et les interventions chirurgicales ne se sont pas allongés.

Multistakeholder Recommendations for Supporting Patients and Families Transitioning From Paediatric to Adult Congenital Heart Disease Care.

Background: Transitioning from paediatric to adult congenital heart disease (CHD) care is a high-risk time for being lost to follow-up. Existing CHD transition programmes have not included patients, caregivers, and health care providers as partners in their development. This study aimed to develop recommendations for a CHD transition programme driven by lived and clinical experiences. Methods: We used a multilevel participatory process that engaged adult and paediatric people living with CHD, their caregivers, and CHD health care providers as members of the research team. We also consulted members of these stakeholder groups through a series of 3 virtual workshops that culminated in the generation of recommendations for the essential components of a CHD transition programme. Results: The Transition Essentials recommendations inform what information, education, or support is required, who should provide it, and when and how it should be provided. Information, education, and support for self-management and knowledge are required for people living with CHD. Caregivers require information, education, and support to build capacity in people living with CHD and navigate their new role in their loved ones' life. The health care team should provide this information, education, and support with peer support options when people living with CHD are 15-22 years of age. This information, education, and support should be individualized, navigate limitations, build over time, have multimodal options, and be available virtually or in person. Conclusions: Engaging those with lived and clinical expertise to develop recommendations for the essential components of a CHD transition programme provides important insights missing from previous studies.

Contexte: La transition des personnes qui vivent avec une cardiopathie congénitale (CC) entre les soins pédiatriques et les soins destinés aux adultes constitue une période où le risque de perte de vue est élevé. Les programmes de transition existants n'ont pas été élaborés avec la participation des patients, des aidants ou des fournisseurs de soins de santé. La présente étude visait à mettre en place des recommandations fondées sur la réalité des personnes concernées et sur l'expérience clinique pour les programmes de transition en contexte de CC. Méthodologie: Nous avons fait appel à un processus participatif à plusieurs niveaux dans lequel des enfants et des adultes vivant avec la CC, des aidants et des fournisseurs de soins de santé du domaine de la CC ont été impliqués comme membres de l'équipe de recherche. Nous avons également mené des consultations auprès de ces groupes d'intervenants dans une série de trois ateliers virtuels qui ont mené à la rédaction de recommandations sur les composantes essentielles d'un programme de transition pour les personnes vivant avec une CC. Résultats: Les recommandations portant sur les impératifs d'une transition réussie énoncent les renseignements, la formation et le soutien nécessaires ainsi que les intervenants qui devraient les offrir, de quelle façon et à quel moment. Les personnes qui vivent avec une CC ont besoin de renseignements, de formation et de soutien pour l'autoprise en charge et l'accès aux connaissances. Quant aux aidants, ils ont aussi besoin de renseignements, de formation et de soutien pour mieux outiller les personnes qui vivent avec une CC et pour mieux comprendre leur nouveau rôle dans la vie de leur proche. Il conviendrait que les professionnels de la santé soient ceux qui offrent ces ressources, lesquelles devraient être personnalisées, tenir compte des lacunes à combler, être cumulatives, offrir des options multimodales et être accessibles en personne ou virtuellement. Les personnes de 15 à 22 ans qui vivent avec une CC devraient également avoir la possibilité de s'entraider. Conclusions: La participation des personnes qui ont une expertise ancrée dans la réalité et une expertise clinique afin de formuler des recommandations sur les éléments essentiels d'un programme de transition pour les personnes qui vivent avec une CC a permis d'obtenir des renseignements intéressants qui ne se trouvaient pas dans les études antérieures.

The Aortic Team Model and Collaborative Decision Pathways for the Management of Complex Aortic Disease: Clinical Practice Update From the Canadian Cardiovascular Society/Canadian Society of Cardiac Surgeons/Canadian Society for Vascular Surgery/Canadian Association for Interventional Radiology.

Disease of the aortic arch, descending thoracic, or thoracoabdominal aorta necessitates dedicated expertise across medical, endovascular, and surgical specialties. Cardiologists, cardiac surgeons, vascular surgeons, interventional radiologists, and others have expertise and skills that aid in the management of patients with complex aortic disease. No specialty is uniformly expert in all aspects of required care. Because of this dispersion of expertise across specialties, an aortic team model approach to decision-making and treatment is advocated. A nonhierarchical partnership across specialties within an interdisciplinary aortic clinic ensures that all treatment options are considered and promotes shared decision-making between the patient and all aortic experts. Furthermore, regionalization of care for aortic disease of increased complexity assures that the breadth of treatment options is available and that favourable volume-outcome ratios for high-risk procedures are maintained. An awareness of best practice care pathways for patient referrals for preventative management, acute care scenarios, chronic care scenarios, and pregnancy might facilitate a more organized management schema for aortic disease across Canada and improve lifelong surveillance initiatives.

Feasibility of MRI assessment of maternal-fetal oxygen transport and consumption relative to maternal position in healthy late gestational pregnancies.

Late gestational supine positioning reduces maternal cardiac output due to inferior vena caval (IVC) compression, despite increased collateral venous return. However, little is known about the impact of maternal position on oxygen (O2 ) delivery and consumption of the gravid uterus, fetus, placenta and lower limbs. We studied the effects of maternal positioning on these parameters in 20 healthy pregnant subjects at 36 ± 2 weeks using magnetic resonance imaging (MRI); a follow-up MRI was performed 6-months postpartum (n = 16/20). MRI techniques included phase-contrast and T1/T2 relaxometry for blood flow and oximetry imaging, respectively. O2 transport was measured in the following vessels (bilateral where appropriate): maternal abdominal descending aorta (DAoabdo ), IVC, ovarian, paraspinal veins (PSV), uterine artery (UtA) and external iliacs, and umbilical. Maternal cardiac output was measured by summing DAothoracic and superior vena cava flows. Supine mothers (n = 6) had lower cardiac output and O2 delivery in the DAoabdo , UtA and external iliac arteries, and higher PSV flow than those in either the left (n = 8) or right (n = 6) lateral positions during MRI. However, O2 consumption in the gravid uterus, fetus, placenta and lower limbs was unaffected by maternal positioning. The ratio of IVC/PSV flow decreased in supine mothers while ovarian venous flow and O2 saturation were unaltered, suggesting a major route of pelvic venous return unaffected by maternal position. Placental-fetal O2 transport and consumption were similar between left and right lateral maternal positions. In comparison to non-pregnant findings, DAoabdo and UtA O2 delivery and pelvic O2 consumption increased, while lower-limb consumption remained constant , despite reduced external iliac artery O2 delivery in late gestation. KEY POINTS: Though sleeping supine during the third trimester is associated with an increased risk of antepartum stillbirth, the underlying biological mechanisms are not fully understood. Maternal cardiac output and uteroplacental flow are reduced in supine mothers due to inferior vena caval compression from the weight of the gravid uterus. This MRI study provides a comprehensive circulatory assessment, demonstrating reduced maternal cardiac output and O2 delivery (uteroplacental, lower body) in supine compared to lateral positioning; however, O2 consumption (gravid uterus, fetus, placenta, lower limbs) was preserved. Unlike other mammalian species, the ovarian veins conduct substantial venous return from the human pregnant uterus that is unaffected by maternal positioning. Lumbar paraspinal venous flow increased in supine mothers. These observations may have important considerations during major pelvic surgery in pregnancy (i.e. placenta percreta). Future studies should address the importance of maternal positioning as a potential tool to deliver improved perinatal outcomes in pregnancies with compromised uteroplacental O2 delivery.

Anesthetic Considerations for Adult Patients With Both Down Syndrome and Congenital Heart Disease Undergoing Noncardiac Surgery: A Review Article.

The population of adult patients with both Down syndrome and congenital heart disease is increasing due to better medical and surgical care. This cohort presents with multiple unique comorbidities, secondary to both progressions of health conditions associated with the aging of patients with Down syndrome, as well as due to pathophysiologic effects of uncorrected, corrected, or palliated congenital heart disease. These patients need frequent medical care and interventions requiring multiple anesthetics. This review focuses on the various factors relevant to the perioperative medical care of adult patients with both Down syndrome and congenital heart disease, founded on existing literature. Various anesthetic considerations for the different patterns of noncardiac and cardiac comorbidities are reviewed, and a systematic approach for the perioperative anesthetic management of these patients is presented.

Practice Patterns in the Management of Pressure Restrictive Perimembranous Ventricular Septal Defects: A Multinational Survey.

Indications for the closure of pressure restrictive perimembranous ventricular septal defects (pmVSD) are not well established in the pediatric population. We sought to assess practice variability among pediatric cardiologists in the United States (US), Canada, Australia, and New Zealand. A survey ascertaining practice patterns, including case vignettes with incremental progression of disease severity, was designed and administered through representative professional cardiac organizations and email listservs in the designated countries. Among the 299 respondents, 209 (70.0%) were from the US, 65 (21.7%) were from Canada and 25 (8.3%) were from Australia and New Zealand. Indications for pressure restrictive pmVSD closure included the presence of left ventricular (LV) dilation for 81.6% (244/299) (defined as z-score ≥ 2 for 59.0% (144/244) and ≥ 3 for 40.2% (98/244)) and significant pulmonary-systemic flow ratio (QP:QS) for 71.2% (213/299) [defined as ≥ 1.5:1 for 36.2% (77/213) and ≥ 2 for 62% (132/213)]. US pediatric cardiologists elected to close restrictive pmVSD at lower LV z-score and QP:QS ratio cut-offs (p-value 0.0002 and 0.013, respectively). In a case vignette, 63.6% (173/272) chose to intervene if there was right coronary cusp prolapse with stable mild aortic regurgitation. Of the remaining cardiologists, 93% (92/99) intervened if the aortic regurgitation was progressive (from trivial to mild). Commonly identified indications with variable thresholds for closure of pressure restrictive pmVSDs included the presence or progression of LV dilation, significant volume loading, and aortic valve prolapse with regurgitation. US pediatric cardiologists may have a lower threshold for pmVSD closure.

Canadian Cardiovascular Society 2022 Guidelines for Cardiovascular Interventions in Adults With Congenital Heart Disease.

Interventions in adults with congenital heart disease (ACHD) focus on surgical and percutaneous interventions in light of rapidly evolving ACHD clinical practice. To bring rigour to our process and amplify the cumulative nature of evidence ACHD care we used the ADAPTE process; we systematically adjudicated, updated, and adapted existing guidelines by Canadian, American, and European cardiac societies from 2010 to 2020. We applied this to interventions related to right and left ventricular outflow obstruction, tetralogy of Fallot, coarctation, aortopathy associated with bicuspid aortic valve, atrioventricular canal defects, Ebstein anomaly, complete and congenitally corrected transposition, and patients with the Fontan operation. In addition to tables indexed to evidence, clinical flow diagrams are included for each lesion to facilitate a practical approach to clinical decision-making. Excluded are recommendations for pacemakers, defibrillators, and arrhythmia-directed interventions covered in separate designated documents. Similarly, where overlap occurs with other guidelines for valvular interventions, reference is made to parallel publications. There is a paucity of high-level quality of evidence in the form of randomized clinical trials to support guidelines in ACHD. We accounted for this in the wording of the strength of recommendations put forth by our national and international experts. As data grow on long-term follow-up, we expect that the evidence driving clinical practice will become increasingly granular. These recommendations are meant to be used to guide dialogue between clinicians, interventional cardiologists, surgeons, and patients making complex decisions relative to ACHD interventions.

The Critical Transfer From Paediatrics to Adult Care in Patients With Congenital Heart Disease: Predictors of Transfer and Retention of Care.

Background: Congenital heart disease is the most common congenital birth defect and presents with differing degrees of complexity. Patients require lifelong specialized care. The transfer from paediatric to adult care is a time of risk that may result in lapses or loss of care. A successful transfer from paediatric to adult care is integral for improved patient outcomes. Methods: In this retrospective study, we used the paediatric cardiology database and the electronic records at the adult congenital heart disease (ACHD) clinic to identify referrals and successful transfer between 2008 and 2017. Successful transfer was defined as a patient referred to the ACHD clinic who was seen in the clinic and has ongoing follow-up. We also sought to identify predictors of a successful transfer. Results: A total of 555 patients were referred to the ACHD clinic (2008-2017). Of all patients referred, 62% had a successful transfer and an ongoing specialist care. The remaining 38% either did not show for first appointments or missed 3 consecutive visits. Independent predictors of a successful transfer were the presence of moderate or complex ACHD, residing within the city limits, older age at the time of referral, and a more recent year of referral. Conclusions: Over one-third of patients did not achieve successful transfer, namely attendance at first clinic visit plus early retention in care. We were able to identify several variables that predict successful transfer. Further research is required to identify interventions that can be implemented to reduce lapses in patient care.

Contexte: La cardiopathie congénitale, qui est la malformation congénitale la plus courante, présente divers degrés de complexité. Les patients qui en sont atteints nécessitent des soins spécialisés tout au long de leur vie. La transition entre les soins pédiatriques et les soins pour adultes est un moment risqué qui peut occasionner un relâchement ou une interruption des soins. Le succès de ce transfert des soins pédiatriques aux soins pour adultes est essentiel à l'amélioration des résultats pour les patients. Méthodologie: Pour cette étude rétrospective, nous avons utilisé la base de données de cardiologie pédiatrique et les dossiers électroniques de la clinique de cardiopathie congénitale de l'adulte (CCA) pour relever les cas de réorientation et de transfert réussi survenus entre 2008 et 2017. On entendait par « transfert réussi ¼ le fait qu'un patient orienté vers la clinique de CCA ait été vu en consultation à la clinique et qu'il fasse l'objet d'un suivi. Nous avons aussi cherché à identifier les facteurs prédictifs d'un transfert réussi. Résultats: Au total, 555 patients ont été orientés à la clinique de CCA entre 2008 et 2017. Chez 62 % de tous ces patients orientés, le transfert a été réussi et les soins spécialisés ont été poursuivis. Les patients représentant les 38 % restants ne se sont pas présentés soit à leur premier rendez-vous, soit à trois visites subséquentes consécutives. Les facteurs prédictifs indépendants du transfert réussi étaient la présence d'une CCA modérée ou complexe, le fait de résider à l'intérieur des limites de la ville, un âge plus avancé au moment de la réorientation et le caractère plus récent de la réorientation. Conclusions: Chez plus du tiers des patients, le transfert n'a pas été réussi, c'est-à-dire que ces patients ne se sont pas présentés à leur première visite à la clinique et que leurs soins n'ont pas été poursuivis rapidement après le transfert. Nous avons pu cerner plusieurs variables qui prédisent les transferts réussis. D'autres recherches seront nécessaires pour trouver les interventions à mettre en œuvre pour réduire les interruptions dans les soins aux patients.

Population and Resource Utilization Among Patients With Adult Congenital Heart Disease: A Snapshot View of a Moderate-Size Canadian Regional Centre.

BACKGROUND: Health care resource utilization for patients with adult congenital heart disease (ACHD) has not been well characterized outside of large Canadian specialized regional centres. We sought to describe the ACHD population and resource utilization patterns seen in a medium regional Canadian centre providing specialized ACHD care. METHODS: A cross-sectional retrospective study was done from a sample of patients seen in 2018 at the ACHD clinic in Manitoba, Canada. Demographic data were collected along with cardiac anatomy and repair type. Health care resource utilization, clinic visits, hospital admissions, unexpected hospital presentations, and cardiac interventions were measured over a 5-year period. RESULTS: A random sample of 262 patients was selected from our specialized ACHD clinic. Mean age was 33.5 (±13.7) years; 48% of the population was female, and >50% resided within the major city limits. A total of 21% of the population had simple anatomy, 44% had moderate anatomy, and 35% had complex anatomy. The most commonly used imaging modality was echocardiography, followed by cardiac magnetic resonance imaging, with more frequent imaging done in patients with complex anatomy. Unexpected hospital encounters occurred at a rate of 16 per 100 person-years. Total inpatient hospital days occurred at a rate of 33 per 100 person-years, and visits to the congenital clinic occurred at a rate of 90 per 100 person-years. CONCLUSIONS: Health care resource utilization appears to be highest in older adults and those with more complex ACHD anatomy. As the overall cohort of adults with ACHD continues to age, resource needs are likely to increase.

CONTEXTE: L'utilisation des ressources de santé à l'extérieur des grands centres spécialisés régionaux au Canada n'a pas été bien caractérisée pour les patients atteints de cardiopathie congénitale de l'adulte (CCA). Nous avons cherché à décrire la population atteinte de CCA et les schémas d'utilisation des ressources observés dans un centre régional canadien de taille moyenne fournissant des soins spécialisés en CCA. MÉTHODOLOGIE: Une étude transversale rétrospective a été réalisée à partir d'un échantillon de patients rencontrés en 2018 dans une clinique spécialisée en CCA du Manitoba, au Canada. Des données démographiques ont été recueillies de même que des données sur l'anatomie cardiaque et le type de réparation. L'utilisation des ressources de soins de santé, les visites à la clinique, les admissions à l'hôpital, les consultions hospitalières imprévues et les interventions cardiaques ont été mesurées sur une période de cinq ans. RÉSULTATS: Un échantillon aléatoire de 262 patients a été sélectionné dans notre clinique spécialisée en CCA. L'âge moyen des patients était de 33,5 (±13,7) ans; 48 % de la population était de sexe féminin et plus de 50 % résidait dans les limites d'une grande ville. Au total, 21 % de la population présentait une anatomie cardiaque simple, 44 % une anatomie modérément complexe et 35 % une anatomie complexe. La technique d'imagerie la plus utilisée était l'échocardiographie, suivie de l'imagerie par résonance magnétique cardiaque, l'imagerie étant plus fréquente chez les patients présentant une anatomie cardiaque complexe. Le taux de consultations hospitalières imprévues était de 16 pour 100 années-patient. Le nombre total de jours d'hospitalisation était de 33 pour 100 années-patient, et le nombre de visites à la clinique spécialisée en CCA était de 90 pour 100 années-patient. CONCLUSIONS: L'utilisation des ressources de soins de santé semble être plus élevée chez les adultes plus âgés et chez ceux qui présentent une anatomie plus complexe liée à la CCA. Puisque l'ensemble de la cohorte d'adultes atteints de CCA continue de vieillir, les besoins en ressources sont susceptibles d'augmenter.

Maternal and Fetal Hemodynamic Adaptations to Pregnancy and Clinical Outcomes in Maternal Cardiac Disease.

BACKGROUND: Although insufficient maternal cardiac output (CO) has been implicated in poor outcomes in mothers with heart disease (HD), maternal-fetal interactions remain incompletely understood. We sought to quantify maternal-fetal hemodynamics with the use of magnetic resonance imaging (MRI) and explore their relationship with adverse events. METHODS: Pregnant women with moderate or severe HD (n = 22; mean age 32 ± 5 years) were compared with healthy control women (n = 21; 34 ± 3 years). An MRI was performed during the third trimester at peak output (maternal-fetal) and 6 months postpartum with return of maternal hemodynamics to baseline (reference). Phase-contrast MRI was used for flow quantification and was combined with T1/T2 relaxometry for derivation of fetal oxygen delivery/consumption. RESULTS: Third-trimester CO and cardiac index (CI) measurements were similar in HD and control groups (CO 7.2 ± 1.5 vs 7.3 ± 1.6 L/min, P = 0.79; CI 4.0 ± 0.7 vs 4.3 ± 0.7 L/min/m,2P = 0.28). However, the magnitude of CO/CI increase (Δ, peak pregnancy - reference) in the HD group exceeded that in the control group (CO 46 ± 24% vs 27 ± 16% [P = 0.007]; CI 51 ± 28% vs 28 ± 17% [P = 0.005]). Fetal growth and oxygen delivery/consumption were similar between groups. Adverse cardiovascular outcomes (nonmutually exclusive) in 6 HD women included arrhythmia (n = 4), heart failure (n = 2), and hypertensive disorder of pregnancy (n = 1); premature delivery was observed in 2 of these women. The odds of a maternal cardiovascular event were inversely associated with peak CI (odds ratio 0.10, 95% confidence interval 0.001-0.86; P = 0.04) and Δ,CI (0.02, 0.001-0.71; P = 0.03). CONCLUSIONS: Maternal-fetal hemodynamics can be well characterised in pregnancy with the use of MRI. Impaired adaptation to pregnancy in women with HD appears to be associated with development of adverse outcomes of pregnancy.

Pulmonary arterial hypertension in pregnancy-a systematic review of outcomes in the modern era.

Pregnancy is hazardous with pulmonary arterial hypertension, but maternal mortality may have fallen in recent years. We sought to systematically evaluate pulmonary arterial hypertension and pregnancy-related outcomes in the last decade. We searched for articles describing outcomes in pregnancy cohorts published between 2008 and 2018. A total of 3658 titles were screened and 13 studies included for analysis. Pooled incidences and percentages of maternal and perinatal outcomes were calculated. Results showed that out of 272 pregnancies, 214 pregnancies advanced beyond 20 gestational weeks. The mean maternal age was 28 ± 2 years, mean pulmonary artery systolic pressure on echocardiogram was 76 ± 19 mmHg. Etiologies include idiopathic pulmonary arterial hypertension (22%), congenital heart disease (64%), and others (15%). Majority (74%) had good functional class I/II. Only 48% of women received pulmonary arterial hypertension-specific therapy. Premature deliveries occur in 58% of pregnancies at mean of 34 ± 1 weeks, most (76%) had Cesarean section. Maternal mortality rate was 12% overall (n = 26); even higher for idiopathic pulmonary arterial hypertension etiology alone (20%). Reported causes of death included right heart failure, cardiac arrest, pulmonary arterial hypertension crises, pre-eclampsia, and sepsis; 61% of maternal deaths occur at 0-4 days postpartum. Stillbirth rate was 3% and neonatal mortality rate was 1%. In conclusion, pulmonary arterial hypertension in pregnancy continues to be perilous with high maternal mortality rate. Continued prospective studies are needed.

Outcomes in Young Adults With Tetralogy of Fallot and Pulmonary Annular Preserving or Transannular Patch Repairs.

BACKGROUND: Early surgical tetralogy of Fallot (ToF) repair involved patching across the pulmonic annulus (transannular patch [TAP] repair), which resulted in severe pulmonic regurgitation. Long-term outcome improvements were anticipated with modifications that preserved the pulmonic annulus (annulus-preserving [AP] repair). The objective of the present study was to evaluate the need for late reintervention in adults with AP repair and those with TAP repair. METHODS: We conducted a retrospective review of adults (born 1981-1996) with childhood intracardiac ToF repairs at a tertiary care center. The primary cardiovascular outcome was need for reintervention after primary intracardiac repair of ToF. Secondary outcomes included a composite of death, heart failure, and ventricular arrhythmias. RESULTS: Two hundred thirty adults were included: 104 with AP repair and 126 with TAP repair. The median age at last follow up was 25 years (interquartile range [IQR] 20-28) and the median follow-up duration was 7.9 years (IQR 3.5-12). Reintervention of any type was significantly more common in the TAP group during both childhood and adulthood (72.2% TAP vs 20.2% AP, HR 5.5, 95% CI 3.4-9.0; P < 0.001). Pulmonary valve replacement (PVR) was almost 6 times more likely in adults with TAP repair (65.1% TAP vs 16.3% AP, HR 5.7, 95% CI 3.4-9.7; P < 0.001). CONCLUSIONS: Patients who had AP ToF repair had significantly fewer late reinterventions compared with TAP repair, with the majority of reinterventions due to PVR. More long-term follow-up is required.