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New York MTA creates task force to secure LIRR, Metro-North rights of way

More news from 2/1/2007.

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  • Transportation

New DOT work plan includes $32 million for U.S. 41/SR 54 interchange

  • C.T. Bowen Times staff

LAND O'LAKES — Twenty-one months ago, the Florida Department of Transportation said it was hitting the brakes on planning improvements at a traffic-clogged intersection in central Pasco.

Instead, the DOT said it would await recommendations from Pasco County and a citizens task force before advancing a proposed fix for the intersection of U.S. 41 and State Road 54. The DOT pause, at the county's request, came after public objections to a planned flyover elevating SR 54 above north-south traffic on U.S. 41.

Now, the state is putting its foot back on the gas, even though the citizens task force has yet to finalize a recommendation. DOT's tentative five-year work program, dated Oct. 17, includes nearly $32 million to buy right of way for a new interchange at SR 54/U.S. 41 beginning in 2022.

"Interesting,'' said Pasco Commissioner Kathryn Starkey. "Actually, that's good. I guess they're starting to plan for something there, even if we don't know what it is.''

The enthusiasm wasn't universal, particularly among Land O'Lakes residents who have advocated that the DOT pursue a no-build alternative.

"They're going to do whatever they want to do because they already have a plan,'' said Christie Zimmer, a Land O'Lakes real estate agent and a member of the citizens task force.

DOT regional spokeswoman Kris Carson said the specific dollar amount, listed at $31,937,100 in the tentative work plan, is "a conservative amount programmed in the last year of the work program, so this gives the county plenty of time to conduct outreach and get the community's vision for this area.''

The dollar amount can go up or down or be shifted for a different purpose if the county recommends the no-build option, Carson said.

The citizens task force, assembled by the Pasco Metropolitan Planning Organization of county commissioners and elected city officeholders, has been considering a dozen options for the intersection, including doing nothing. Five of the proposals include elevating SR 54 for vehicles and/or mass transit above U.S. 41. Other ideas call for at-grade improvements to the intersection and adding frontage roads.

Preliminary estimates for right-of-way costs range from as little as $1.7 million (building four elevated lanes in the existing right of way) up to $110 million for what is known as a parallel flow intersection in which left-turning cars bypass the intersection via frontage roads.

The MPO put together the task force after severe community push-back against a 2014 proposal from a private company to build and operate an elevated toll road along the State Road 54/56 corridor between U.S. 19 and Bruce B. Downs Boulevard. The DOT pulled the plug on that idea when the company said it needed public subsidies in order for the $2 billion project to work.

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Amid the citizens group's work, the DOT also put forth the idea of a flyover taking SR 54 traffic over U.S. 41, a project that could cost as much as $180 million and affect two dozen businesses, two homes and a county fire station. The Central Pasco Chamber of Commerce opposed the plan, and 15 of 19 speakers at a Land O'Lakes public hearing in December 2015 also objected. Two months later, the DOT agreed to wait for the task force recommendations.

The right-of-way money is separate from a planned short-term fix to extend turn lanes for eastbound motorists on SR 54, allowing through traffic easier access to the intersection. That work is projected to be done in 2019 at a cost of about $1 million.

Approximately 99,000 vehicles pass through the U.S. 41/SR 54 intersection daily, and growth projections call for daily traffic to increase to 208,000 vehicles over the next 25 years.

A flyover at the intersection has been discussed for more than two decades after a community uproar killed a planned east-west highway through Lutz to connect the Veterans Expressway and Interstate 75. Later, the state built a new interchange at I-75 and SR 56 and widened both SR 54 and 56 to six lanes, effectively turning that stretch of highway into the de facto east-west route.

"I'm glad they're starting to plan something,'' said Starkey. "There has to be a decision made. We can't continue the way it is.''

Hillsborough County Reporter

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DPWH Creates Right-of-Way Task Force for Urgent Infrastructure Projects

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A task force that will handle right-of-way acquisition has been created by the Department of Public Works and Highways (DPWH) to minimize delay in project implementation.

Under Department Order No. 203, series of 2016, DPWH Secretary Mark A. Villar ordered the creation of Unified Project Management Office Right-of-Way (UPMO-ROW) Task Force that will now handle the monitoring of ROW issues that affect the implementation of UPMO road, bridge and flood control projects nationwide.

The UPMO-ROW Task Force, headed by DPWH Undersecretary for UPMO operations Emil K. Sadain, with Legal Service Director Estrella T. Decena-Zaldivar as vice chairman and UPMO Cluster directors as members, will monitor the ROW acquisition status and recommend appropriate actions for ROW issues.

To support the UPMO-ROW Task Force, a Technical Working Group (TWG), to be composed of representatives of UPMO Cluster Directors, will also be created. The TWG is responsible for the screening and verification of documents submitted for IROW claims as well as the accuracy of the computation of land valuations and disturbance compensation.

Based on the same guidelines, the payment of ROW claims shall only be processed by the concerned District Engineering Offices once the revalidation of submitted documents is made by the UPMO-ROW Task Force.

With the creation of new task force, the processing of ROW claims for UPMO projects will speed  up, resulting to a more effective and expeditious completion of urgent infrastructure. (DPWH)

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DoT may set up task force to implement right of way rules in states

‘Right of way’ rules pertain to regulatory clearances needed by a telecom and infrastructure service provider to lay out infrastructure like telecom towers and optic fibre cables.

right of way task force

The right of way rules were notified on November 15, 2016 but most states are yet to implement them. The rules called for every state to appoint a nodal officer for facilitating securing of clearances from various authorities.

Related stories

That Sinking Feeling: Still gripped by economic crisis, Pakistan grapples with deepening political uncertainties

A project for installing towers in Delhi may need clearances from one or more of the 5 municipalities, Delhi Development Authority, Delhi Metro Rail Corp, the Delhi Pollution Control Committee, Airport Authority of India and others.

The right of way rules call for establishing an automated process for submission of an application. As per the rules, the appropriate authority has to approve or reject within 60 days of a service provider submitting an application to lay the infrastructure. Permission is deemed granted if there is no decision on it within 60 days.

While most states are yet to appoint a nodal officer, Rajasthan has included right of way in its policy while Haryana has in-principle agreed on it, the official said.

Right of way is a major stumbling block for telecom companies in laying out their infrastructure as securing clearances from various local bodies and authorities is a long and cumbersome process.  This is the last thing a company, struggling with irate customers suffering from call drops, wants.

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Understanding Municipal Rights-of-Way: From Centerline to Edge (Part 1)

September 27, 2017  by  Oskar Rey Category:  Streets and Sidewalks

Understanding Municipal Rights-of-Way: From Centerline to Edge (Part 1)

MRSC gets a lot of inquiries about street rights-of-way. Over the years, we have touched on specific aspects of rights-of-way on our website and in blog articles. In this two part series, I will try to cover the fundamentals of rights-of-way and the duties of local governments and abutting owners. This article will cover right-of-way fundamentals and the issues presented by use of rights-of-way for travel. The next article will cover sidewalks, utilities, trees and unused portions of the right-of-way.   

Fundamentals: What is a right-of-way and what can be done with it?

It is a common misconception that rights-of-way are owned by local government. In fact, the general rule is that city or county rights-of-way are easements for public travel and other secondary street purposes (such as utilities). This is the reason why streets, when vacated, generally become the property of the abutting owners to the centerline of the right-of-way. Here are links to more detailed information on the nature of right-of-way and street vacation . 

Despite its easement status, municipalities have broad authority to manage and regulate the rights-of-way. That authority stems from the police power under the Washington State Constitution (Article XI, Section 11), as well as statutes such as RCW 35A.11.020 , which provides code cities with broad power “in regard to the acquisition, sale, ownership, improvement, maintenance, protection, restoration, regulation, use, leasing, disposition, vacation, abandonment or beautification of public ways.” Other municipal entities also have broad authority: counties ( RCW 36.75.050 ); first class cities ( RCW 35.22.280 (7) and (8)); second class cities ( RCW 35.23.440 (33)); and towns ( RCW 35.27.370 (4)).

Washington cases have observed that the primary purpose of a right-of-way is public travel. However, municipalities may authorize other incidental uses of rights-of-way, including private uses, which do not unreasonably interfere with public travel. See, e.g., Winkenwerder v. City of Yakima , (upholding ordinance allowing placement of advertising on top of parking meters). The circumstances under which such incidental uses may be authorized is a legislative decision for the municipality, not a judicial question. Additional information can be found on MRSC’s Right-of-Way Use Permit webpage. 

Use of right-of-way for travel—municipal duties

Rendering of street with pothole in front of house

Sometimes dangerous conditions, such as a potholes (see Figure 1), may occur in a street. If the dangerous condition was not caused by the municipality, the municipality may still be liable for it if: (a) it has notice of the dangerous condition; and (b) reasonable opportunity to correct it. O’Neill v. City of Port Orchard . Notice of the dangerous condition may be actual or constructive. Actual knowledge may occur when a city crew observes a dangerous condition in the right-of-way or when a citizen reports it to the city. “Constructive knowledge” means that a municipality should have known of a dangerous condition, even if it did not have actual knowledge. In some cases, constructive knowledge may be inferred from the amount of time a dangerous condition exists prior to an accident. Nguyen v City of Seattle .

Rendering of house with vegetation obstructing view at intersection

Municipalities should have procedures for documenting and responding to dangerous right-of-way conditions in timely fashion. With respect to vegetation, many municipalities use “trim letters” to inform abutting property owners of their responsibility to keep the right-of-way free from sight obstructions. Based on Wuthrich and the speed with which blackberries grow in this region, municipalities should also monitor and take necessary action in areas where vegetation may create sight obstructions.

Please stay tuned for Part 2 of this blog series!

Questions? Comments?

If you have questions about rights-of-way or other local government issues, please use our  Ask MRSC form  or call us at  (206) 625-1300  or  (800) 933-6772 . If you have comments about this blog post or other topics you would like us to write about, please  email me directly.

MRSC is a private nonprofit organization serving local governments in Washington State. Eligible government agencies in Washington State may use our free, one-on-one Ask MRSC service to get answers to legal, policy, or financial questions.

Photo of Oskar Rey

About Oskar Rey

Oskar Rey is an attorney with Ogden Murphy Wallace PLLC. Prior to this he worked as a legal consultant at MRSC from 2016 to 2024. Oskar has practiced municipal law since 1995 and served as Assistant City Attorney for the City of Kirkland from 2005 to 2016, where he worked on a wide range of municipal topics, including land use, public records, and public works.

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Pakistans First Right Of Way Policy: Towards Digital Pakistan

  • June 3, 2021
  • Author: Fareha Iqtidar Khan

Related Research Program: Mapping Emerging and Future Technologies

Read Full Document

Key Points:

  • Following initial rejection and backdoor diplomacy, the Public & Private Right of Way Policy Directive was finally approved in January 2021 for facilitating the installation and maintenance of telecom infrastructure throughout the country.
  • The Prime Ministers Task Force on IT & Telecom originally sought to devise specific rules for the Right of Way (RoW). However, given the absence of unanimity amongst the stakeholders, the task force presented a basic framework instead, in the form of a policy directive.
  • RoW provides an authorisation enabling operators to set up telecom installations on roads, railways, electric transmission cables, et cetera following a payment.
  • Key provisions of the policy include one-window operation, fee determination, dispute resolution, ensuring national security, guarding against health hazards, declaring telecom infrastructure as critical infrastructure, sharing of RoW, and establishing common service corridors.
  • The implementation of the newly introduced policy directive is expected to reduce the administrative roadblocks for the telecom firms.
  • RoW policy is deemed to be a baseline for Digital Pakistan by providing the framework for 5G readiness, ease of doing business, and improved connectivity of small and medium enterprises (SMEs).
  • Although the current policy does provide a sufficient framework towards digitalisation yet ensuring its implementation can prove to be an uphill task given its non-binding nature.
  • The policy has not specified a target amount of monetary savings or an established period to acquire its goals. Consequently, there is no way to determine or gauge the extent of effectiveness of its different provisions.

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Fareha Iqtidar Khan

Fareha Iqtidar Khan serves as a Senior Associate Editor at the Centre for Strategic and Contemporary Research. Holding an MPhil in International Relations from the National Defence University, she also occasionally teaches at esteemed public sector universities.

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Harris slams 'politically motivated' report as Biden to name task force to protect classified docs

WASHINGTON — Vice President Kamala Harris on Friday slammed the report by a Justice Department special counsel into Joe Biden’s mishandling of classified documents that raised questions about the president’s memory, calling it “politically motivated” and “gratuitous,” as the White House said the president would take steps to safeguard classified materials during presidential transitions.

The report from Robert Hur , the former Maryland U.S. Attorney selected by Attorney General Merrick Garland to investigate Biden found evidence that Biden willfully held onto and shared with a ghostwriter highly classified information, but laid out why he did not believe the evidence met the standard for criminal charges, including a high probability that the Justice Department would not be able to prove Biden’s intent beyond a reasonable doubt.

The White House has said Biden erred in having the documents in his home and Ian Sams, a spokesperson for the White House counsel’s office, said on Friday that Biden would soon name a task force “to ensure that there are better processes in place” to protect classified materials when administrations change.

The Hur report described the 81-year-old Democrat’s memory as “hazy,” “fuzzy,” “faulty,” “poor” and having “significant limitations.” It noted that Biden could not recall defining milestones in his own life such as when his son Beau died or when he served as vice president.

Asked whether the White House would release a copy of the transcript of Biden’s interview with Hur that could dispute Hur’s characterizations, Sams said parts of it were classified, but that if parts of it could be declassified, “we’ll take a look at that and make a determination.”

Taking a question from a reporter at the conclusion of a gun violence prevention event at the White House, Harris said that as a former prosecutor, she considered Hur’s comments “gratuitous, inaccurate, and inappropriate.”

She noted that Biden’s two-day sit-down with Hur occurred just after the Oct. 7 attack by Hamas on Israel, where more than 1,200 people were killed and about 250 were taken hostage — including many Americans.

“It was an intense moment for the commander in chief of the United States of America,” Harris said, saying she spent countless hours with Biden and other officials in the days that followed and he was “on top of it all.”

She added that “the way that the president’s demeanor in that report was characterized could not be more wrong on the facts and clearly politically motivated, gratuitous.”

Harris concluded saying a special counsel should have a “higher level of integrity than what we saw.”

Her comments came a day after Biden insisted that his “memory is fine.” and grew visibly angry at the White House, as he denied forgetting when his son died. Beau Biden died of brain cancer in 2015 at the age of 46.

Sams suggested that the political environment led Hur, who was appointed as U.S. attorney by former President Donald Trump , to include the comments. “There’s an environment that we are in, that generates a ton of pressure, because you have congressional Republicans, other Republicans, attacking prosecutors that they don’t like,” he said.

right of way task force

  • Case report
  • Open access
  • Published: 10 February 2024

Detection of gene mutation in the prognosis of a patient with arrhythmogenic right ventricular cardiomyopathy: a case report

  • Dinh Phong Phan 1 , 2 ,
  • Tuan Viet Tran 1 , 2 ,
  • Vo Kien Le 1 &
  • Tuan Viet Nguyen 2  

Journal of Medical Case Reports volume  18 , Article number:  49 ( 2024 ) Cite this article

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Arrhythmogenic right ventricular cardiomyopathy (ARVC), or more recently known as arrhythmogenic cardiomyopathy (ACM), is an heritable disorder of the myocardium characterized by progressive fibrofatty replacement the heart muscle and risk of ventricular arrhythmias and sudden cardiac death (SCD). We report a case study to demonstrate the role of gene mutation detection in risk stratification for primary prevention of SCD in a young patient diagnosed with ARVC.

Case presentation

A 15-year-old Asian (Vietnamese) male patient with no history of documented tachyarrhythmia or syncope and a family history of potential SCD was admitted due to palpitations. Clinical findings and work-up including cardiac magnetic resonance imaging (MRI) were highly suggestive of ARVC. Gene sequencing was performed for SCD risk stratification, during which PKP2 gene mutation was found. Based on the individualized risk stratification, an ICD was implanted for primary prevention of SCD. At 6 months post ICD implantation, the device detected and successfully delivered an appropriate shock to terminate an episode of potentially fatal ventricular arrhythmia. ICD implantation was therefore proven to be appropriate in this patient.

Conclusions

While gene mutations are known to be an important factor in the diagnosis of ARVC according to the 2010 Task Force Criteria and recent clinical guidelines, their role in risk stratification of SCD remains controversial. Our case demonstrated that when used with other clinical factors and family history, this information could be helpful in identifying appropriate indication for ICD implantation.

Peer Review reports

Arrhythmogenic right ventricular cardiomyopathy (ARVC), or more recently known as arrhythmogenic cardiomyopathy (ACM), is an heritable disorder of the myocardium characterized by progressive myocardial changes and risk of ventricular arrhythmias and sudden cardiac death (SCD). First described in the late 1970s and early 1980s [ 1 ], histopathological characterization of the right ventricle (RV) identifies multiple changes, most notably the presence of progressive fibrofatty or fat replacement of the myocardium, leading to RV dilatation and dysfunction [ 2 ]. This process forms heterogeneous zones of the myocardium correlated with arrhythmogenic substrates, which trigger the occurrence of ventricular tachycardias (VT) and ventricular fibrillation (VF) that can lead to SCD [ 3 , 4 ]. However, recent studies have shown that biventricular involvement is more prevalent than previously thought and therefore, the term AVRC is being replaced by arrhythmogenic cardiomyopathy (ACM) [ 5 ] It has been reported that 73% of ARVC index patients carry mutations in genes encoding the desmosomal proteins [ 6 , 7 ] necessary for the maintenance of stable intercellular connections. The prevalence of these gene mutations ranges from 28 to 58% [ 8 , 9 ]. TGFB3, RYR2, TTN, TNEM43, DES, DSP, PKP2, DSG2, DSC2, JUP, PLN, LMNA, SCN5A , and CTNNA3 have all been identified as playing a role in the pathogenesis of ARVC in recent studies [ 10 ]. This finding provides the foundation for the latest recommendation in European Society of Cardiology (ESC) guidelines as well as Heart Rhythm Society (HRS) consensus to consider gene mutations as a criterion for ARVC diagnosis and risk stratification [ 11 , 12 ]. Nevertheless, the role of gene mutations in guiding treatment decisions remains controversial, particularly in the risk stratification of primary prevention of SCD [ 13 ]. We report a case study to demonstrate the role of gene mutation detection in risk stratification for treatment decisions.

A 15-year-old Asian (Vietnamese) male high school student as well as basketball player presented with the first-ever episode of palpitations. He had never had syncope or presyncope, documented arrhythmia or been diagnosed with cardiovascular diseases. His 27-year-old brother had died suddenly one year ago due to out of hospital cardiac arrest. Most likely cause of death was identified as arrhythmia by emergency physicians after other differentials such as cerebral vascular stroke, myocardial infarction, congenital and acquired cardiac disease were excluded by imaging, blood tests. On examination , his vital signs were normal with no clinical signs of heart failure. His electrocardiography (ECG) on admission (Fig.  1 ) showed frequent and various forms of premature ventricular complexes (PVC) with negative T waves in leads V1–V3.

figure 1

Electrocardiography on admission showing baseline rhythm was sinus rhythm, T wave inversion in leads V1–V3, with frequent and polymorphic premature ventricular complexes

Transthoracic echocardiography showed mildly dilated right ventricle with no other significant structural or functional abnormalities. The 24-h ECG ambulatory (Fig.  2 ) revealed significant PVC burden, accounting for 10.5% of the total number of heart beats. At least three distinct PVC morphologies were observed. There were occasionally coupled PVCs but no non-sustained or sustained VT was captured.

figure 2

24-h ambulatory Electrocardiography: two consecutive premature ventricular complexes variants were recorded

Electrophysiology (EP) study was indicated for two purposes: (1) to ablate the frequent PVCs, and (2) to conduct programmed electrical stimulation (PES) to induce sustained ventricular arrhythmias for risk stratification. During the procedure, frequent PVCs with various patterns were observed. These PVCs originated from the RV at different sites of the postero-lateral wall, proximal to the tricuspid annulus. Multiple attempts of mapping and radiofrequency ablation failed to terminate all PVCs. PES delivered at the RV apex with two extra stimuli induced non-sustained episodes of VT repeatedly (Fig.  3 ).

figure 3

Programmed electrical stimulation induced a non-sustained ventricular tachycardia

Cardiac Magnetic Resonance Imaging (MRI) showed enlarged RV with dyskinesia and reduced ejection fraction (RV ejection fraction 34.97%). An aneurysm close to the RV apex was also observed. Late-enhancement signal with Gadolinium showed diffused fatty infiltration in the RV free wall (Fig.  4 ). Left ventricular structure and function was normal (LV ejection fraction 62%).

figure 4

Cardiac Magnetic Resonance Imaging showed intramyocardial late gadolinium enhancement due to diffused fibrofatty scar involving the RV free wall

We then performed molecular-genetic analysis using next-generation sequencing (NGS) methods to identify gene mutations for further risk stratification. A mutation in the PKP2 gene which encodes Plakophilin-2 protein was identified (Fig.  5 ). The most prevalent cause of ARVC is a heterozygous mutation in this gene. So far, more than 200 mutations in the PKP2 gene have been documented, the majority of which are point mutations.

figure 5

The results of molecular-genetic analysis

Finally, patient was diagnosed with ARVC according to the Modified Task Force criteria for ARVC proposed by the International Task Force of ESC and International Society and Federation of Cardiology in 2010. The specific diagnostic criteria were:

Major criterion: cardiac MRI found RV dyskinesia and RVEF 34.97%.

Major criterion: T wave inversion in leads V1–V3 on ECG

Minor criterion: 9899 PVCs on Holter ECG.

Major criterion: the PKP2 gene mutation.

After a thorough consideration of all risk factors as well as careful discussion with the patient’s parents, we decided to implant an implantable cardioverter defibrillator (ICD) for primary prevention of SCD. Patient was initially discharged on beta-blocker (bisoprolol 5 mg daily). After one month, amiodarone 100 mg daily was started when ICD check found many episodes of non-sustained VT.

Follow-up and outcome

At 6 months follow-up, the patient experienced a spontaneous episode of fast VT or VF, a potentially fatal ventricular arrhythmia, which was successfully terminated by an ICD shock (Fig.  6 ).

figure 6

ICD detected ( A ) and delivered an appropriate shock ( C ) to terminate a fast VT or VF after a failed ATP (antitachycardia pacing) attempt ( B )

ARVC is a heritable cardiomyopathy which may cause life-threatening ventricular arrhythmias leading to SCD. Fibrofatty replacement of RV myocardium remains to be the main histopathological characteristic of the disease [ 1 , 2 ]. In 2010, the Task Force Criteria (TFC) for clinical diagnosis of the ARVC based on multiple factors was proposed by experts in the field of heart failure and cardiomyopathy. In 2019, HRS published an Expert Consensus Statement on Evaluation, Risk Stratification, and Management of Arrhythmogenic Cardiomyopathy, in which ACM is defined as the disease in heart muscle that involves the RV, left ventricle, or both [ 12 ]. The pathophysiological feature is fibrofatty infiltration of myocardium which may predispose patients to life-threatening arrhythmias and ventricular dysfunction. ACM are classified into 3 phenotypic variants including the classic ARVC, ALVC (arrhythmogenic left ventricular cardiomyopathy) and the disease involving both the ventricles. The diagnostic criteria for ARVC variant were based on major and minor criteria of the 2010 TFC [ 19 ]. Based on these diagnostic criteria, our patient met the diagnosis of definite ARVC.

In the 2010 TFC, gene mutation is considered as one of the major diagnostic criteria in the family history section. Specifically, identification of a pathogenic or likely pathogenic ACM mutation in the patient under evaluation is categorized as one of the major diagnostic criteria. Genetic analysis of our patient reveals the presence of a mutation of the PKP2 gene, which has been shown to be one of the mutations potentially related to ARVC.

The gene mutation in this patient changes a single nucleotide at the position NM_001005242.3: c.1379-2A > G belongs to the PKP2 gene’s intron region. We used Clinvar and Varsome tool to characterize the mutation and applied the American College of Medical Genetics and Genomics (ACMG) criteria to determine its pathogenicity [ 14 ]. Based on these databases, this mutation belongs to a category of mutations that have the potential to cause ARVC. PKP2  mutations are also the most common cause of ARVC in some populations. The  PKP2  gene encodes the synethis of a protein called plakophilin 2, which makes up structures called desmosomes. These structures form junctions that attach cells to one another. Abnormalities in the binding protein trigger myocardial remodeling and fat replacement process which may lead to arrthymia. PKP2 mutations play an important role in the pathogenesis and progression of ARVC [ 15 , 16 ].

According to the findings of Judith et al. the mutation detection prevalence in ARVC patients was 63%, with PKP2 mutations accounted for 46% of all mutations [ 17 ]. In addition, a study on 90 ARVC patients in China by Jingru Bao et al. [ 18 ] revealed 57 subjects (63%) having genetic mutations, 58% of which occurred in the PKP2 gene. Furthermore, the study’s findings demonstrated that ARVC patients with a gene mutation had a higher risk of VT than those without mutation. Similar finding was also observed in patients with and without PKP2 gene mutation. This suggests that gene mutations, even if occurring in a single-gene, especially the PKP2 gene, can be a predictor of the risk of cardiovascular events associated with ventricular arrhythmias and sudden death.

SCD due to ventricular arrhythmias can be prevented by ICD implantation [ 11 ]. Expert consensus has recommended that in ARVC patients, ICD is indicated for secondary prevention in patients with aborted cardiac arrest or hemodynamically unstable sustained VT, and for primary prevention in patients who have high risk of ventricular arrhythmias and SCD [ 12 ]. As our patient had no history of aborted SCD, ventricular arrhythmias associated syncope, sustained VT, or severely reduced LV ejection fraction on admission, risk factors for ventricular arrhythmias should be assessed to decide whether ICD implantation was necessary. According to the risk stratification guidelines recommended by the 2019 HRS Expert Consensus Statement on Evaluation, Risk Stratification, and Management of Arrhythmogenic Cardiomyopathy, our patient met only four minor risk factors for ventricular arrhythmias (male sex, > 1000 PVCs/24 h, RV dysfunction [34.97% on cardiac MRI] and proband status). Accordingly, the indication for primary prevention with an ICD for this patient was a class IIb recommendation with the level of evidence B. However, considering his family history with potential SCD in his older brother, the result of gene mutation and presuming that the disease’s severity may progress in the future, and with the family’s preference, a decision was made to implant an ICD for primary prevention of SCD. At 6 months post implantation, the patient experienced palpitations and near-syncope. A sustained fast VT or VF, a potentially fatal ventricular arrhythmia with an average cycle length of 229 ms, was detected and successfully terminated by an ICD shock. The decision of ICD implantation was finally proven to be appropriate for this patient.

Gene mutations are known to be an important factor in the diagnosis of ARVC according to TFC 2010 criteria and later clinical guidelines. Although the role of gene mutation in risk stratification remains controversial, it still plays an important role in individualized risk stratification and management. In this case, gene mutation, along with other clinical factors and family history, was proven to be helpful in guiding treatment decisions of ICD implantation for primary prevention of SCD in a young patient diagnosed with ARVC.

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The data supporting this study is with the author and has been included within the manuscript.

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Phong Phan Dinh was a major contributor in writing the manuscript. Tuan Viet Tran and Tuan Viet Nguyen coordinated the writing and managed the submission of the manuscript. Phong Phan Dinh, Vo Kien Le, Tuan Viet Tran were the main physicians in charge of providing care and implantation the ICD to the patient. All authors read and approved the final manuscript.

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Phan, D.P., Tran, T.V., Le, V.K. et al. Detection of gene mutation in the prognosis of a patient with arrhythmogenic right ventricular cardiomyopathy: a case report. J Med Case Reports 18 , 49 (2024). https://doi.org/10.1186/s13256-023-04326-w

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