HMV is an essential intervention for pediatric patients with chronic respiratory failure resulting from a range of underlying conditions. By providing long-term ventilatory support, HMV helps maintain gas exchange, reduces the need for extended hospital stays, and improves overall quality of life for both children and their families [2,3,11]. Neuromuscular disorders, such as Duchenne muscular dystrophy (DMD), spinal muscular atrophy, and congenital myopathies, are some of the most common indications for HMV. These conditions result in progressive respiratory muscle weakness, causing hypoventilation, recurrent infections, and eventual respiratory failure. HMV prevents nocturnal hypoventilation, delays daytime hypercapnia, and reduces the risk of acute respiratory failure in these patients, allowing for improved survival and quality of life [12,13]. Chronic pulmonary conditions, including severe BPD, cystic fibrosis, and advanced interstitial lung disease, also warrant the use of HMV. In patients with BPD, HMV supports lung growth and development, reduces work of breathing, and decreases oxygen dependency. Similarly, in patients with cystic fibrosis, HMV alleviates airway obstruction, reduces hypercapnia, and delays disease progression, improving both pulmonary function and life expectancy [6,12,14]. Central respiratory drive impairments, such as those seen in CCHS, traumatic brain injury, or post-encephalitic states, represent another major indication for HMV. These children rely on ventilatory support, particularly during sleep, to prevent life-threatening hypoventilation episodes. In addition, chest wall abnormalities, such as severe kyphoscoliosis or thoracic dystrophy, restrict lung expansion and reduce functional residual capacity, necessitating HMV to relieve hypoventilation and improve daily functioning.

Post-surgical recovery often requires temporary or long-term ventilatory support. Children recovering from procedures such as diaphragmatic repair, cardiac surgery, or spinal fusion benefit from HMV to stabilize respiratory mechanics and prevent complications during the recovery phase. These diverse indications highlight the critical role of HMV in managing a wide spectrum of pediatric respiratory conditions [6,10,12,14-16].

The safe, effective, and sustainable implementation of HMV in pediatric patients requires careful consideration of clinical stability, home environment readiness, caregiver capabilities, and ethical aspects of care.

The choice of ventilation mode in HMV must be tailored to the specific respiratory mechanics and functional limitations of the child. Pressure-controlled ventilation is widely used in pediatric HMV, particularly for children with neuromuscular disorders characterized by fluctuating lung compliance. By maintaining constant airway pressure during inspiration, pressure-controlled ventilation reduces the risk of ventilator-induced lung injury, making it a preferred mode for patients with weak respiratory muscles and variable lung mechanics.

Volume-controlled ventilation, in contrast, delivers a fixed tidal volume regardless of changes in lung compliance or resistance. This mode is especially advantageous for children with restrictive lung diseases, such as severe scoliosis or interstitial lung disease, where precise tidal volume delivery is essential for maintaining adequate ventilation. However, younger children may experience discomfort due to pressure fluctuations during breath delivery. This limitation can be addressed with hybrid modes or advanced ventilators equipped with adaptive algorithms to improve synchronization [14,19,20].

For children capable of partial respiratory effort, pressure support ventilation provides an effective means of augmenting spontaneous breaths. By delivering inspiratory pressure support, pressure support ventilation reduces the work of breathing and supports more natural breathing patterns. This mode is particularly beneficial for older children who retain some degree of respiratory drive but require assistance to achieve adequate tidal volumes. Similarly, spontaneous/timed modes, which combine spontaneous breathing support with timed backup breaths, are frequently employed in conditions such as CCHS, where intermittent respiratory effort requires support during periods of apnea. Advanced modes, such as volume-assured pressure support, combine the benefits of both pressure and volume control. Volume-assured pressure support guarantees a minimum tidal volume while dynamically adjusting pressure levels to improve patient-ventilator synchronization and comfort. This hybrid approach is particularly effective for patients with mixed respiratory pathologies, such as those with both restrictive and obstructive components [21]. For children with obstructive sleep apnea, continuous positive airway pressure is often used to maintain airway patency during sleep, preventing upper airway collapse and ensuring uninterrupted ventilation [2,14,17,19].

The selection of ventilator modes is undoubtedly important; however, the parameter settings within each mode can have an even greater impact on the patient’s respiratory status. For example, in infants with BPD, ventilator dys-synchrony can exacerbate hyperinflation and desaturation, while significantly increasing the work of breathing. Therefore, achieving optimal synchrony with the ventilator is critically important. For this reason, the precise adjustment of individual parameters, such as respiratory rate and sensitivity is essential. In the application of non-invasive ventilation (NIV), while other parameter settings are important, the adjustment of positive end-expiratory pressure is particularly critical, proper positive end-expiratory pressure settings are essential to overcome upper airway resistance and ensure effective airflow into the lungs. No consensus exists regarding the selection of ventilator type. However, the primary goal is to reduce the work of breathing and provide optimal respiratory support. Consequently, the decision is often based on the clinician’s expertise and preference.

The selection of an appropriate interface is equally important, as it serves as the critical connection between the ventilator and the patient. Interfaces are broadly categorized into non-invasive and invasive types, each with unique advantages and limitations that must be considered based on the child’s clinical condition and needs. Non-invasive interfaces, such as nasal masks, oronasal masks, and total face masks, are commonly used in children who do not require continuous airway access. Nasal masks are often preferred for their comfort and ease of use, particularly in younger children. However, their effectiveness can be compromised by air leaks if the child’s mouth remains open during use, necessitating additional measures such as chin straps or sealing modifications. Oronasal masks, which cover both the nose and mouth, offer more stable ventilation in children who are mouth breathers during sleep but may cause discomfort or pressure sores on the nasal bridge with prolonged use. Total face masks, which cover the entire face, are an alternative for children with nasal irritation or frequent skin breakdown, although their size and weight may limit tolerability, particularly in younger patients [12,22-24].

Invasive interfaces, such as tracheostomy tubes, are indicated for children requiring high levels of ventilatory support, continuous airway access, or assistance with secretion management due to severe bulbar dysfunction. Tracheostomy tubes provide reliable ventilation by minimizing air leaks, and the choice between cuffed and uncuffed tubes depends on the clinical scenario. Cuffed tubes are preferred in settings requiring high-pressure ventilation, while uncuffed tubes are often used in younger children to reduce the risk of tracheal damage [3,12,14,20,22].

The selection of ventilation modes and interfaces must be tailored to the child’s developmental stage, underlying condition, respiratory drive, and tolerance for invasive or non-invasive support. Regular reassessment is critical, as children’s needs evolve with growth and changes in their clinical condition. For example, children with progressive conditions such as DMD may require periodic adjustments to their ventilatory settings to accommodate declining respiratory capacity [2,3,19]. Advances in ventilator technology have significantly increased the adaptability and efficacy of HMV. Features such as leak compensation algorithms, adaptive pressure adjustments, and advanced monitoring capabilities enable better synchronization between the patient and the ventilator. Single-limb circuits with leak ports are commonly used in NIV for their simplicity and effectiveness in managing leaks. In contrast, double-limb circuits, which allow precise measurement of inspiratory and expiratory volumes, are preferred in invasive ventilation scenarios. By carefully selecting and regularly reassessing ventilation modes and interfaces, clinicians can optimize the delivery of HMV, ensuring effective respiratory support while minimizing complications and making patients more comfortable. This individualized approach is essential for achieving therapeutic goals and improving the quality of life for pediatric patients and their families [2,3,19,22].

Clinical monitoring focuses on assessing respiratory function, growth, and development. Evaluations of gas exchange, including nocturnal oxygen saturation and CO₂ levels, are crucial for detecting hypoventilation, particularly during sleep. Polysomnography is recommended to optimize ventilator settings, especially for children with conditions such as CCHS or obstructive sleep apnea. These evaluations help prevent long-term complications and refine ventilator support [1,11,25,26]. Monitoring growth and development is equally important, as children on HMV often face challenges related to their underlying conditions, such as increased caloric demands or swallowing difficulties in neuromuscular disorders. Poor growth may signal inadequate nutritional intake or suboptimal ventilatory support. Anthropometric measurements and dietary assessments, along with consultations with nutritionists, ensure that children achieve developmental milestones [1,11,26].

Ensuring that the ventilator meets the patient’s evolving needs requires periodic reviews of its performance. Key parameters, such as tidal volume, respiratory rate, and pressure settings, must be regularly assessed. Advanced ventilators equipped with monitoring systems enable caregivers and clinicians to identify issues such as patient-ventilator asynchrony, excessive air leaks, or suboptimal pressure levels. Reviewing ventilator logs provides valuable insights into therapy adherence, particularly for older children who may resist wearing masks during sleep [11]. Routine maintenance of ventilatory equipment is vital to prevent device-related complications. Filters, circuits, and humidifiers should be replaced according to the manufacturer’s recommendations, and the functionality of ventilators should be tested regularly. For children dependent on invasive ventilation, tracheostomy care, including cleaning, suctioning, and monitoring for infections or obstructions, is essential for minimizing risks [7,10].

The objectives of HMV extend beyond maintaining adequate gas exchange and include improving quality of life, reducing hospital admissions, and promoting integration into family and community life. Follow-up visits should assess the child’s overall health, frequency of respiratory infections, and hospitalization rates. Changes in symptoms or increased hospitalizations may signal the need for ventilatory adjustments or additional management of comorbidities like gastroesophageal reflux or airway infections. Psychosocial assessments are also critical, as the demands of caregiving can lead to emotional stress and financial strain. Periodic evaluations of caregiver well-being, along with access to mental health resources and support services, help sustain effective home-based care. Providing respite care and encouraging participation in peer support groups further alleviates the caregiver’s burden and promotes the family’s well-being [26].

The frequency of follow-up visits depends on the patient’s stability and care complexity. Initially, frequent visits are required to assess ventilatory effectiveness, monitor the child’s adaptation, and address any challenges faced by caregivers. As the child’s condition stabilizes, follow-up intervals may be extended to every 3-6 months, with additional visits scheduled as needed for emerging health concerns [10,11,26].

Emergency preparedness is a vital component of HMV follow-up care. Families must have access to backup power supplies, spare ventilator circuits, and emergency contact numbers for their healthcare team. Caregivers should be trained to recognize early signs of respiratory distress, such as increased work of breathing, cyanosis, or altered mental status, and respond effectively to emergencies like ventilator failure or acute illness. Simulation-based training and emergency drills increase caregivers’ confidence and preparedness to manage crises [18,27].

The decision to initiate weaning is guided by evidence of stabilization or improvement in the underlying condition requiring HMV. Children with reversible conditions, such as BPD or Guillain-Barré syndrome, are often suitable candidates for successful weaning as they recover or mature. Conversely, children with progressive diseases, such as DMD or advanced spinal muscular atrophy, may not be candidates for complete weaning due to the ongoing deterioration of respiratory function.

Indicators of readiness for weaning include stable oxygenation and carbon dioxide levels, reduced ventilatory dependence, improved respiratory muscle strength, and normalized nocturnal gas exchange as demonstrated by polysomnographic assessments. These indicators are crucial for identifying children who can safely tolerate reduced ventilatory support, particularly at night when the risk of hypoventilation is highest [2,28].

Weaning from HMV follows a stepwise approach designed to allow the child’s respiratory system to adapt gradually. For children dependent on invasive ventilation via tracheostomy, the process often begins with daytime trials of spontaneous breathing using tracheostomy collars or T-pieces. During these trials, clinicians monitor respiratory rates, oxygenation, and carbon dioxide levels to assess tolerance. If successful, ventilatory parameters, such as inspiratory pressure or backup rates, are gradually reduced. Once daytime weaning is achieved, capping the tracheostomy or using speaking are options to further decrease ventilatory reliance while promoting upper airway functionality. For children receiving NIV, the process typically involves reducing pressure support or transitioning to less intensive modes, such as continuous positive airway pressure, before complete discontinuation. Nocturnal weaning is approached cautiously to minimize the risks of hypoventilation, with close monitoring and frequent reassessments. Outpatient-based weaning strategies supported by structured follow-up have proven effective in settings with robust healthcare infrastructure and caregiver education [28].

Continuous monitoring during the weaning process is essential to ensure that the child maintains stable gas exchange and does not experience respiratory distress. Key parameters include oxygen saturation, carbon dioxide levels (end-tidal or transcutaneous), respiratory rate, and signs of increased work of breathing, such as accessory muscle use or retractions. Blood gas analyses provide additional confirmation of ventilatory adequacy, particularly during advanced stages of weaning. Polysomnography is a critical tool during nocturnal weaning, providing data on hypoventilation, apneas, and overall sleep quality. These evaluations allow clinicians to adjust ventilator settings and ensure the safety of reduced ventilatory support during sleep. Frequent monitoring and reassessments enable timely interventions to address potential setbacks and optimize the weaning process [3,28].

The weaning process may be hindered by physiological and psychological factors. Persistent medical challenges, such as chronic lung disease, airway abnormalities (e.g., tracheomalacia or subglottic stenosis), and insufficient respiratory muscle strength, can limit the ability to sustain independent breathing. Additionally, structural changes associated with prolonged tracheostomy dependence can complicate the process, requiring specialized interventions.

Psychological and behavioral barriers also play a significant role, particularly in older children who have relied on ventilatory support for extended periods. Such children may develop psychological dependence on the ventilator, associating it with comfort and security. Overcoming these barriers requires a multidisciplinary approach involving respiratory therapists, psychologists, and caregivers to build the child’s confidence and gradually transition them toward independence [12,13,28].

Successful weaning from HMV provides substantial benefits, including reduced dependence on medical equipment, increased mobility, and greater participation in daily and social activities, such as attending school and engaging in recreational pursuits. Families also benefit from a decreased caregiving burden and reduced financial strain associated with long-term ventilatory support. The success of weaning depends on several factors, including the underlying condition, the timing of the intervention, and the availability of a coordinated care plan. Children with reversible conditions are more likely to achieve complete independence from mechanical ventilation. In contrast, those with chronic or progressive diseases may achieve partial weaning, such as limiting ventilatory support to nocturnal use. Individualized care plans, frequent reassessments, and multidisciplinary collaboration are essential to optimizing outcomes for each child [2,3].

By carefully tailoring the weaning strategies to each child’s needs, providing continuous monitoring, and addressing both physiological and psychological barriers, weaning from HMV enables children to transition toward greater independence. This approach not only increases respiratory autonomy but also improves the overall quality of life for pediatric patients and their families.

Medical risks are primarily linked to complications from ventilatory equipment and underlying conditions. For tracheostomy-dependent children, risks include infections, airway obstructions, and tracheal stenosis, necessitating routine care and monitoring. Children using NIV face issues such as pressure sores, skin breakdown, and midface hypoplasia, particularly with poorly fitted masks. Inadequate humidification can lead to mucosal dryness and airway resistance, compromising ventilation efficiency. Recurrent respiratory infections and impaired respiratory drive in ventilator-dependent children further complicate care, underscoring the need for regular clinical evaluation [1,3,11,22].

Equipment malfunction, such as sensor errors or power outages, poses a critical threat, potentially leading to hypoventilation or respiratory failure. Insufficient caregiver expertise in managing ventilatory systems exacerbates these risks. Regular maintenance, proper programming of ventilator settings, and caregiver training are essential to mitigate complications like atelectasis and pneumothorax [1,3,11,19].

Children on HMV may experience social isolation, reduced participation in activities, and emotional distress, particularly adolescents coping with stigma. Caregivers face physical exhaustion, emotional stress, and financial strain due to their constant responsibilities, including equipment monitoring and emergency management. Comprehensive support systems, including counseling, respite care, and financial assistance, are critical to address these challenges and sustain caregiving efforts [5,13,26,29]..

Ethical dilemmas arise when the burden of care outweighs potential benefits, requiring decisions about initiating or withdrawing HMV. Legal risks include inadequate training, delayed emergency response, or equipment failure. Transparent communication, shared decision-making, and adherence to ethical principles help families make informed choices, while institutional safeguards minimize legal risks [1,7,13,14,30].

Caregivers face round-the-clock responsibilities, including operating ventilatory equipment, tracheostomy care, suctioning, and monitoring for respiratory distress. The need for constant vigilance, especially with nocturnal ventilation, often disrupts sleep, leading to chronic fatigue and negatively affecting physical health. Emotional stress is compounded by anxiety over potential emergencies, social isolation, and financial strain from out-of-pocket expenses for equipment and caregiving support. These pressures frequently lead to caregiver burnout and can disrupt family dynamics, with siblings feeling neglected and caregivers struggling to balance multiple roles [14,17].

Comprehensive education programs are critical for reducing caregivers’ burden and improving HMV management. Training should begin during hospitalization and continue post-discharge, covering ventilator operation, alarm troubleshooting, tracheostomy care, airway clearance, and emergency protocols. Simulation-based training builds caregiver confidence by allowing them to practice responses to realistic scenarios. Teaching caregivers to recognize early signs of respiratory distress and maintain ventilatory equipment reduces hospitalizations and infection risks. Education should also include strategies for managing stress and avoiding burnout, with access to support groups and respite care reinforcing caregivers’ ability to sustain their roles [10,18].

Ongoing support and access to resources are essential for maintaining HMV at home. Regular follow-ups with healthcare teams address caregiver concerns and reinforce training. Telemedicine provides remote consultations and troubleshooting, reducing the need for frequent hospital visits. Financial assistance programs alleviate economic burdens, while instructional materials, such as step-by-step guides and online modules, support caregivers in managing day-to-day challenges [14,18].

Effective caregiver education improves clinical outcomes for children and reduces caregiver stress. Confident, well-trained caregivers are better equipped to prevent complications and respond to emergencies, reducing hospital readmissions and associated costs. Strong partnerships between caregivers and healthcare providers, characterized by open communication and ongoing support, empower families to deliver safe and effective care. This collaboration improves the quality of life for both children and their caregivers while ensuring the long-term sustainability of HMV in the home [10,13].