Aortopathy in Congenital Heart Disease in Adults: Aortic Dilatation with Decreased Aortic Elasticity that Impacts Negatively on Left Ventricular Function

Koichiro Niwa 대한심장학회 2013 Korean Circulation Journal Vol.43 No.4

Bicuspid aortic valve and/or coarctation of the aorta are consistently associated with ascending aortic and para-coarctation medial ab-normalities. Medial abnormalities in the ascending aorta are prevalent in other types of patients with a variety of forms congenital heart disease (CHD), such as single ventricle, persistent truncus arteriosus, transposition of the great arteries, hypoplastic left heart syndrome, te-tralogy of Fallot. These abnormalities encompass a wide age range, and may predispose to dilatation, aneurysm, and rupture that necessi-tates aortic valve and root surgery. This dilatation can develop in CHD patients without stenotic region. These CHDs exhibit ongoing dilatation of the aortic root and reduced aortic elasticity and increased aortic stiffness that may relate to intrinsic properties of the aortic root. The concept of aortic dilatation is shifting a paradigm of aortic dilatation, as so called post stenotic dilatation, to primary intrinsic aortopahy. These aortic dilatation and increased stiffness can induce aortic aneurysm, rupture of the aorta and aortic regurgitation, but also provoke left ventricular hypertrophy, reduced coronary artery flow and left ventricular failure. We can recognize this association of aortic pathophysiological abnor-mality, aortic dilation and aorto-left ventricular interaction as a new clinical entity: “aortopathy”.

Adult Congenital Heart Disease with Pregnancy

Koichiro Niwa 대한심장학회 2018 Korean Circulation Journal Vol.48 No.4

The number of women with congenital heart disease (CHD) at risk of pregnancy is growing because over 90% of them are grown-up into adulthood. The outcome of pregnancy and delivery is favorable in most of them provided that functional class and systemic ventricular function are good. Women with CHD such as pulmonary hypertension (Eisenmenger syndrome), severe left ventricular outflow stenosis, cyanotic CHD, aortopathy, Fontan procedure and systemic right ventricle (complete transposition of the great arteries [TGA] after atrial switch, congenitally corrected TGA) carry a high-risk. Most frequent complications during pregnancy and delivery are heart failure, arrhythmias, bleeding or thrombosis, and rarely maternal death. Complications of fetus are prematurity, low birth weight, abortion, and stillbirth. Risk stratification of pregnancy and delivery relates to functional status of the patient and is lesion specific. Medication during pregnancy and post-delivery (breast feeding) is a big concern. Especially prescribing medication with teratogenicity should be avoidable. Adequate care during pregnancy, delivery, and the postpartum period requires a multidisciplinary team approach with cardiologists, obstetricians, anesthesiologists, neonatologists, nurses and other related disciplines. Caring for a baby is an important issue due to temporarily pregnancy-induced cardiac dysfunction, and therefore familial support is mandatory especially during peripartum and after delivery. Timely pre-pregnancy counseling should be offered to all women with CHD to prevent avoidable pregnancy-related risks. Successful pregnancy is feasible for most women with CHD at relatively low risk when appropriate counseling and optimal care are provided.

Metabolic Syndrome in Adult Congenital Heart Disease

Koichiro Niwa 대한심장학회 2019 Korean Circulation Journal Vol.49 No.8

In adult congenital heart disease (ACHD), residua and sequellae after initial repair develop late complications such as cardiac failure, arrhythmias, thrombosis, aortopathy, pulmonary hypertension and others. Acquired lesions with aging such as hypertension, diabetes mellitus, obesity can be negative influence on original cardiovascular disease (CVD). Also, atherosclerosis may pose an additional health problem to ACHD when they grow older and reach the age at which atherosclerosis becomes clinically relevant. In spite of the theoretical risk of atherosclerosis in ACHD due to above mentioned factors, cyanotic ACHDs even after repair are noted to have minimal incidence of coronary artery disease (CAD). Acyanotic ACHD has similar prevalence of CAD as the general population. However, even in cyanotic ACHD, CAD can develop when they have several risk factors for CAD. The prevalence of risk factor is similar between ACHD and the general population. Risk of premature atherosclerotic CVD in ACHD is based, 3 principal mechanisms: lesions with coronary artery abnormalities, obstructive lesions of left ventricle and aorta such as coarctation of the aorta and aortopathy. Coronary artery abnormalities are directly affected or altered surgically, such as arterial switch in transposition patients, may confer greater risk for premature atherosclerotic CAD. Metabolic syndrome is more common among ACHD than in the general population, and possibly increases the incidence of atherosclerotic CAD even in ACHD in future. Thus, ACHD should be screened for metabolic syndrome and eliminating risk factors for atherosclerotic CAD.

The Usefulness of a Three Dimensional Roadmap and Pressure-Wire Assisted Angioplasty in Chronic Thromboembolic Pulmonary Hypertension

Atsushi Mizuno,Koichiro Niwa 대한심장학회 2014 Korean Circulation Journal Vol.44 No.4

IMAGE OF INTEREST : Pocket flashlight-elicited Quincke pulse for aortic dissection diagnosis

( Atsushi Mizuno ),( Koichiro Niwa ) 대한내과학회 2013 The Korean Journal of Internal Medicine Vol.28 No.5

Black Defects in Severe Spontaneous Echo Contrast Teach Us the Protective Aspect of Mitral Regurgitation Visually

Atsushi Mizuno,Nobuhiro Takao,Koichiro Niwa 한국심초음파학회 2013 Journal of Cardiovascular Imaging (J Cardiovasc Im Vol.21 No.1

Endomyocardial Biopsy and Magnetic Resonance Imaging of Acute Myocarditis with Adult-Onset Still’s Disease

Masahiro Yamazoe,Atsushi Mizuno,Yasuhiro Suyama,Yutaro Nishi,Koyu Suzuki,Koichiro Niwa,Masato Okada 대한심장학회 2014 Korean Circulation Journal Vol.44 No.6

A 36-year-old female with a high-grade fever and epigastric abdominal pain was prescribed antibiotics, but developed hypoxia and dyspnea. An echocardiography revealed diffuse hypokinesis and massive pericardial effusion, after which diagnostic cardiac catheterizationand an endomyocardial biopsy (EMB) were peformed to reveal fibrosis and infiltration of inflammation cells composed primarily of neutrophils. Clinical manifestation of a spiking fever, leukocytosis, elevated ferritin levels, skin rash and EMB findings led to a diagnosis of adultonsetStill’s disease (AOSD) with acute myocarditis. Pulse therapy of intravenous methylprednisolone was performed for three days, followedby a daily dose of prednisone (60 mg). After a course of steroid therapy for fever and pericardial effusion, and conducting a leftventricular ejection fraction, the patient showed improvement and was discharged asymptomatic within 32 days of admission. This studyis the first to report on a case of myocarditis in AOSD diagnosed by neutrophil infiltration in the myocardium.

Spinal Sagittal Alignment, Hospital Anxiety and Depression Scale Scores, and Patient-Reported Outcome among People with Sporting Activity

Oe Shin,Yamato Yu,Hasegawa Tomohiko,Yoshida Go,Kobayashi Sho,Yasuda Tatsuya,Banno Tomohiro,Arima Hideyuki,Mihara Yuki,Ushirozako Hiroki,Yamada Tomohiro,Ide Koichiro,Watanabe Yuh,Haruo Niwa,Matsuyama Y 대한척추외과학회 2020 Asian Spine Journal Vol.14 No.3

Study Design: Retrospective cohort study.Purpose: This study aimed to investigate how participation in sporting activity affects patient-reported outcome (PRO), including Neck Disability Index (NDI), in males and females.Overview of Literature: Previously, our study reported that factors with a negative influence on the NDI in females were a lack of sporting activities. However, it was still unclear why it affected poor scores of NDI.Methods: The subjects were 473 volunteers. They were divided into two groups (activity and non-activity) according to participation or non-participation in sporting activities using a self-filled questionnaire. The evaluation items were height, weight, grip strength, bone density, Hospital Anxiety and Depression Scale (HADS) score, standing radiographic parameters, PRO (evaluated by EuroQol-5 dimension [EQ-5D], Oswestry Disability Index [ODI]), and NDI.Results: There were 101 males in the non-activity group and 69 in the activity group and 178 females in the non-activity group and 125 in the activity group. For the males, the evaluation items with significant influence were cervical lordosis (non-activity group:activity group, 17°:22°) and T1 slope minus cervical lordosis (10°:6°, <i>p</i> <0.05). For the females, the evaluation items with significant influence were sagittal vertical axis (28:14 mm), HADS (10.4:8.4), EQ-5D (0.79:0.86), ODI (17:12), and NDI (12:9, <i>p</i> <0.01). HADS and PRO in the females were significantly correlated with the EQ-5D (−0.40), ODI (0.43), and NDI (0.55).Conclusions: Males who participated in sporting activities had better cervical spine alignment but no effect on PRO. Females with sporting activities had better spinal global alignment and less mental stress. It is suggested that sporting activity in females might be associated with PRO because HADS highly correlates with PRO.