A case of congenital left ventricular diverticulum in a patient with autosomal dominant polycystic kidney disease: possible mechanistic link between polycystin and ventricular diverticulum

Seishi Aihara, Shunsuke Yamada, Hidenori Matsusaka, Rami Tashiro, Hideaki Oka, Taro Kamimura, Atsumi Harada, Toshiaki Nakano, Takanari Kitazono, Kazuhiko Tsuruya

Research output: Contribution to journalArticle

Abstract

A 40-year-old woman had been followed as an outpatient to manage chronic kidney disease secondary to autosomal dominant polycystic kidney disease (ADPKD). Atrial premature contraction was found incidentally on an electrocardiogram during her regular follow-up examination. Subsequent transthoracic echocardiography detected an abnormal structure located very close to the left ventricular outflow tract (23 mm long × 15 mm wide in diastole). The structure was finally diagnosed as congenital left ventricular diverticulum (CLVD) using transesophageal echocardiography, contrast-enhanced computed tomography, and magnetic resonance imaging. Although CLVD occasionally causes intraventricular coagulation, lethal arrhythmia, and congestive heart failure, the size and location of her diverticulum remained unchanged over time and a 24-h Holter electrocardiogram showed no lethal arrhythmias. Accordingly, neither anticoagulation therapy nor surgical resection of the diverticulum was performed. To the best of our knowledge, ours is the first case of CLVD in a patient with ADPKD. Because gene abnormalities in polycystin coding are mechanistically related to the development of colonic diverticulum and abnormal cyst formation in ADPKD patients, we suspected that CLVD and abnormal cyst formation were related to the same gene abnormality in ADPKD. More case reports, case series studies, and basic research are required to determine whether CLVD in ADPKD is mechanistically associated with abnormal polycystin or just a coincidence.

Original languageEnglish
Pages (from-to)237-242
Number of pages6
JournalCEN case reports
Volume7
Issue number2
DOIs
Publication statusPublished - Nov 1 2018

Fingerprint

TRPP Cation Channels
Autosomal Dominant Polycystic Kidney
Diverticulum
Cysts
Cardiac Arrhythmias
Electrocardiography
Colon Diverticula
Atrial Premature Complexes
Diastole
Transesophageal Echocardiography
Chronic Renal Insufficiency
Genes
Echocardiography
Outpatients
Heart Failure
Tomography
Magnetic Resonance Imaging

All Science Journal Classification (ASJC) codes

  • Nephrology

Cite this

A case of congenital left ventricular diverticulum in a patient with autosomal dominant polycystic kidney disease : possible mechanistic link between polycystin and ventricular diverticulum. / Aihara, Seishi; Yamada, Shunsuke; Matsusaka, Hidenori; Tashiro, Rami; Oka, Hideaki; Kamimura, Taro; Harada, Atsumi; Nakano, Toshiaki; Kitazono, Takanari; Tsuruya, Kazuhiko.

In: CEN case reports, Vol. 7, No. 2, 01.11.2018, p. 237-242.

Research output: Contribution to journalArticle

@article{8c9f9b308ddf4f758e94a3c2479790bf,
title = "A case of congenital left ventricular diverticulum in a patient with autosomal dominant polycystic kidney disease: possible mechanistic link between polycystin and ventricular diverticulum",
abstract = "A 40-year-old woman had been followed as an outpatient to manage chronic kidney disease secondary to autosomal dominant polycystic kidney disease (ADPKD). Atrial premature contraction was found incidentally on an electrocardiogram during her regular follow-up examination. Subsequent transthoracic echocardiography detected an abnormal structure located very close to the left ventricular outflow tract (23 mm long × 15 mm wide in diastole). The structure was finally diagnosed as congenital left ventricular diverticulum (CLVD) using transesophageal echocardiography, contrast-enhanced computed tomography, and magnetic resonance imaging. Although CLVD occasionally causes intraventricular coagulation, lethal arrhythmia, and congestive heart failure, the size and location of her diverticulum remained unchanged over time and a 24-h Holter electrocardiogram showed no lethal arrhythmias. Accordingly, neither anticoagulation therapy nor surgical resection of the diverticulum was performed. To the best of our knowledge, ours is the first case of CLVD in a patient with ADPKD. Because gene abnormalities in polycystin coding are mechanistically related to the development of colonic diverticulum and abnormal cyst formation in ADPKD patients, we suspected that CLVD and abnormal cyst formation were related to the same gene abnormality in ADPKD. More case reports, case series studies, and basic research are required to determine whether CLVD in ADPKD is mechanistically associated with abnormal polycystin or just a coincidence.",
author = "Seishi Aihara and Shunsuke Yamada and Hidenori Matsusaka and Rami Tashiro and Hideaki Oka and Taro Kamimura and Atsumi Harada and Toshiaki Nakano and Takanari Kitazono and Kazuhiko Tsuruya",
year = "2018",
month = "11",
day = "1",
doi = "10.1007/s13730-018-0335-0",
language = "English",
volume = "7",
pages = "237--242",
journal = "CEN Case Reports",
issn = "2192-4449",
publisher = "Springer Japan",
number = "2",

}

TY - JOUR

T1 - A case of congenital left ventricular diverticulum in a patient with autosomal dominant polycystic kidney disease

T2 - possible mechanistic link between polycystin and ventricular diverticulum

AU - Aihara, Seishi

AU - Yamada, Shunsuke

AU - Matsusaka, Hidenori

AU - Tashiro, Rami

AU - Oka, Hideaki

AU - Kamimura, Taro

AU - Harada, Atsumi

AU - Nakano, Toshiaki

AU - Kitazono, Takanari

AU - Tsuruya, Kazuhiko

PY - 2018/11/1

Y1 - 2018/11/1

N2 - A 40-year-old woman had been followed as an outpatient to manage chronic kidney disease secondary to autosomal dominant polycystic kidney disease (ADPKD). Atrial premature contraction was found incidentally on an electrocardiogram during her regular follow-up examination. Subsequent transthoracic echocardiography detected an abnormal structure located very close to the left ventricular outflow tract (23 mm long × 15 mm wide in diastole). The structure was finally diagnosed as congenital left ventricular diverticulum (CLVD) using transesophageal echocardiography, contrast-enhanced computed tomography, and magnetic resonance imaging. Although CLVD occasionally causes intraventricular coagulation, lethal arrhythmia, and congestive heart failure, the size and location of her diverticulum remained unchanged over time and a 24-h Holter electrocardiogram showed no lethal arrhythmias. Accordingly, neither anticoagulation therapy nor surgical resection of the diverticulum was performed. To the best of our knowledge, ours is the first case of CLVD in a patient with ADPKD. Because gene abnormalities in polycystin coding are mechanistically related to the development of colonic diverticulum and abnormal cyst formation in ADPKD patients, we suspected that CLVD and abnormal cyst formation were related to the same gene abnormality in ADPKD. More case reports, case series studies, and basic research are required to determine whether CLVD in ADPKD is mechanistically associated with abnormal polycystin or just a coincidence.

AB - A 40-year-old woman had been followed as an outpatient to manage chronic kidney disease secondary to autosomal dominant polycystic kidney disease (ADPKD). Atrial premature contraction was found incidentally on an electrocardiogram during her regular follow-up examination. Subsequent transthoracic echocardiography detected an abnormal structure located very close to the left ventricular outflow tract (23 mm long × 15 mm wide in diastole). The structure was finally diagnosed as congenital left ventricular diverticulum (CLVD) using transesophageal echocardiography, contrast-enhanced computed tomography, and magnetic resonance imaging. Although CLVD occasionally causes intraventricular coagulation, lethal arrhythmia, and congestive heart failure, the size and location of her diverticulum remained unchanged over time and a 24-h Holter electrocardiogram showed no lethal arrhythmias. Accordingly, neither anticoagulation therapy nor surgical resection of the diverticulum was performed. To the best of our knowledge, ours is the first case of CLVD in a patient with ADPKD. Because gene abnormalities in polycystin coding are mechanistically related to the development of colonic diverticulum and abnormal cyst formation in ADPKD patients, we suspected that CLVD and abnormal cyst formation were related to the same gene abnormality in ADPKD. More case reports, case series studies, and basic research are required to determine whether CLVD in ADPKD is mechanistically associated with abnormal polycystin or just a coincidence.

UR - http://www.scopus.com/inward/record.url?scp=85061118555&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85061118555&partnerID=8YFLogxK

U2 - 10.1007/s13730-018-0335-0

DO - 10.1007/s13730-018-0335-0

M3 - Article

C2 - 29876750

AN - SCOPUS:85061118555

VL - 7

SP - 237

EP - 242

JO - CEN Case Reports

JF - CEN Case Reports

SN - 2192-4449

IS - 2

ER -