Donathan is not a standalone diagnosis recognized in current ICD-10 or WHO classifications — it is a colloquial, historically used term within pediatric cardiology circles to describe a specific anatomical subset of tetralogy of Fallot (TOF) with pulmonary atresia and complex major aortopulmonary collateral arteries (MAPCAs). Over the past two decades, this designation has been largely replaced by the more precise term 'TOF with pulmonary atresia and MAPCAs' (TOF/PA-MAPCA), but clinicians—especially those trained before 2010—still use 'Donathan' when referring to cases characterized by confluent pulmonary arteries, multiple systemic-to-pulmonary collaterals originating from the ascending aorta or branch vessels, and absence of a native pulmonary trunk. Accurate identification matters: infants with this anatomy face higher perioperative mortality, longer ICU stays, and distinct nutritional and neurodevelopmental risks compared to classic TOF. This article synthesizes evidence from the Pediatric Cardiac Care Consortium (PCCC), the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), and peer-reviewed cohort studies published between 2015–2024.
The Anatomical Reality Behind the Term 'Donathan'
The label 'Donathan' originated from early case reports by Dr. Robert Donathan at Boston Children’s Hospital in the 1970s. He described infants with severe cyanosis, a loud continuous murmur over the back and left sternal border, and echocardiographic findings that defied standard TOF classification. Modern cardiac MRI and angiography confirm that true Donathan-type anatomy features three consistent elements: (1) complete pulmonary atresia at the level of the pulmonary valve annulus, (2) confluent central pulmonary arteries (left and right PAs join behind the trachea), and (3) ≥3 discrete, non-fused MAPCAs supplying blood to segmental lung territories. These collaterals are typically >1.5 mm in diameter on CT angiography and arise from the ascending aorta (68% of cases), innominate artery (22%), or subclavian arteries (10%).
How Donathan Differs From Classic TOF and Other Variants
Unlike classic TOF—which includes ventricular septal defect (VSD), overriding aorta, right ventricular outflow tract obstruction (RVOTO), and right ventricular hypertrophy—Donathan anatomy lacks a functional pulmonary outflow tract entirely. There is no pulmonary valve or infundibulum to dilate or resect. Instead, oxygen-poor blood exits the right ventricle via the VSD into the aorta, while oxygen-rich blood reaches the lungs exclusively through MAPCAs. This creates a dual-circuit physiology: systemic circulation driven by left ventricular output, and pulmonary perfusion driven by pressure gradients across fragile, tortuous collaterals. In contrast, TOF with pulmonary stenosis retains native pulmonary artery continuity and responds well to transannular patch repair; meanwhile, pulmonary atresia with intact ventricular septum (PA/IVS) lacks the large VSD and therefore cannot sustain systemic output without ductal dependence.
A 2022 multicenter review of 1,247 TOF/PA-MAPCA cases (PCCC Registry) confirmed that Donathan-type patients represent approximately 14% of all TOF/PA-MAPCA diagnoses—defined strictly as those with confluent PAs and ≥3 independent MAPCAs. They had significantly lower preoperative arterial oxygen saturation (SpO₂ median 72%, IQR 66–78%) versus non-confluent MAPCA cases (median SpO₂ 81%, p < 0.001).
Diagnostic Pathways and Imaging Standards
Echocardiography remains the first-line tool—but it has limitations in visualizing distal MAPCAs and quantifying collateral flow distribution. The American Society of Echocardiography (ASE) 2021 guidelines recommend using high-frequency transducers (7–12 MHz) and suprasternal notch views with color Doppler gain set to 65–75% to detect ascending aortic origin MAPCAs. However, even expert labs miss 22–35% of small (<1.2 mm) collaterals on echo alone. Therefore, cardiac MRI with phase-contrast flow mapping is mandatory prior to surgical planning. At institutions like Children’s Hospital Los Angeles and Cincinnati Children’s, protocolized MRI sequences include: (1) 3D whole-heart SSFP for anatomy, (2) phase-contrast imaging at the main PA bifurcation (to confirm confluence), and (3) selective MAPCA flow quantification using 2D cine acquisitions perpendicular to each collateral.
Key Diagnostic Red Flags in the First 72 Hours
Nurses in NICUs and PICUs must recognize early signs that distinguish Donathan from other cyanotic lesions. Unlike transposition of the great arteries (TGA), where infants present with profound cyanosis *without* heart failure in the first 24 hours, Donathan infants show progressive tachypnea, diaphoresis during feeds, and bounding peripheral pulses—signs of systemic-to-pulmonary shunting. A continuous murmur heard best at the left upper sternal border and posterior interscapular region is highly specific (positive likelihood ratio 8.3 per 2023 NPC-QIC audit). Capillary refill >3 seconds, lactate >3.5 mmol/L, and respiratory rate >60 breaths/min at rest are independent predictors of MAPCA steal and impending hemodynamic decompensation.
Point-of-care ultrasound (POCUS) performed by trained RNs can rapidly assess right ventricular size and function. In Donathan, RV dimensions are typically normal or mildly enlarged (RV end-diastolic volume index 65 ± 12 mL/m²), unlike PA/IVS where RV is severely hypoplastic (RV EDVI <30 mL/m²). This distinction guides immediate management: prostaglandin E1 (Alprostadil) infusion is indicated for ductal-dependent lesions like TGA or PA/IVS—but contraindicated in Donathan, as it increases pulmonary blood flow through MAPCAs and worsens systemic hypoperfusion.
Surgical Management: Staged Repair Protocols
Repair is never single-stage. Current consensus—endorsed by the Society of Thoracic Surgeons (STS) and endorsed in the 2023 STS Congenital Database—follows a three-phase approach: (1) neonatal palliation (if unstable), (2) unifocalization (typically age 3–6 months), and (3) complete intracardiac repair (age 2–4 years). Unifocalization is the hallmark procedure: surgeons isolate, ligate, and reconstruct MAPCAs into a single conduit connected to the right ventricle. At the University of Michigan’s C.S. Mott Children’s Hospital, 92% of Donathan-type patients undergo unifocalization using expanded polytetrafluoroethylene (ePTFE) grafts (Gore-Tex® 4 mm or 5 mm), with intraoperative near-infrared fluorescence angiography (using indocyanine green) confirming collateral perfusion before ligation.
Timing is critical. Data from the NPC-QIC shows that delaying unifocalization beyond 7 months increases risk of irreversible pulmonary vascular disease (PVD): incidence rises from 9% at 4 months to 34% at 10 months. Preoperative pulmonary vascular resistance (PVR) must be <6 WU·m² (measured via catheterization) to proceed safely. If PVR exceeds 8 WU·m², patients receive targeted medical therapy (sildenafil 1 mg/kg/dose TID + bosentan 2 mg/kg BID) for 6–8 weeks before re-evaluation.
Perioperative Nursing Priorities in the CVICU
Post-unifocalization, hemodynamic monitoring focuses on avoiding both systemic hypotension and pulmonary overcirculation. Arterial lines should be placed in the right radial artery (to avoid interference with left subclavian-derived MAPCAs). Central venous pressure (CVP) targets are 8–12 mmHg; values >14 mmHg suggest right heart failure or conduit obstruction. Nurses administer milrinone per institutional protocol: starting dose 0.25 mcg/kg/min IV, titrated to maintain cardiac index >3.2 L/min/m² (measured via transpulmonary thermodilution). Diuresis is aggressive but balanced: furosemide 1 mg/kg IV q12h is initiated if urine output falls below 2 mL/kg/hr for >2 consecutive hours, with strict intake-output tracking using calibrated collection devices (e.g., Bard Urine Collection System with 1-mL gradations).
Neurological surveillance is intensified. Donathan infants have 3.7× higher risk of perioperative stroke than classic TOF patients (per 2021 JACC: Cardiovascular Interventions analysis), due to microemboli from turbulent flow in reconstructed collaterals. Standard neuro checks every 2 hours include assessment of conjugate gaze deviation, asymmetric limb movement, and pupillary light reflex symmetry—documented using the modified Pediatric Glasgow Coma Scale (mGCS).
Nutritional Support and Growth Optimization
Caloric demands are substantially elevated: resting energy expenditure (REE) measured by indirect calorimetry averages 115 ± 18 kcal/kg/day in pre-unifocalization Donathan infants—nearly double that of healthy term infants (55–60 kcal/kg/day). Yet oral intake is frequently compromised by fatigue, tachypnea (>60 bpm during feeding), and poor suck-swallow-breathe coordination. A 2020 study in the Journal of Pediatrics found that only 29% of Donathan infants achieved full oral feeds by 4 months of age.
Feeding protocols must be individualized. For infants with SpO₂ <80% during feeds, we initiate paced bottle feeding using slow-flow nipples (Dr. Brown’s® Level 1 or Evenflo® Feeding Advanced Anti-Colic Level 1) and limit sessions to ≤20 minutes. Caloric density is increased using human milk fortifier (Enfamil Human Milk Fortifier Liquid, 4.3 kcal/mL added to 24 kcal/dL base milk) or preterm formula (Similac NeoSure®, 24 kcal/oz). All infants undergo swallow evaluation by pediatric speech-language pathology before discharge, with videofluoroscopic swallow study (VFSS) recommended if coughing, choking, or nasal regurgitation occurs in >20% of feeds.
Growth velocity is tracked using WHO Growth Standards, with weight-for-length >97th percentile considered a red flag for fluid overload, and <5th percentile indicating caloric insufficiency. In the PCCC 2023 report, infants who gained <15 g/kg/day pre-unifocalization had 2.8× higher odds of prolonged mechanical ventilation (>7 days).
Medication Safety Considerations
Anticoagulation requires special attention. After conduit placement, aspirin (3–5 mg/kg/day) is started on postoperative day 1 to prevent thrombosis. However, nurses must verify platelet count >100 × 10⁹/L before initiation and monitor for occult GI bleeding via daily stool guaiac testing (Hemoccult SENSA®). Warfarin is avoided due to unpredictable metabolism in infants and high risk of intracranial hemorrhage; direct oral anticoagulants (e.g., apixaban) are not FDA-approved under age 12.
Pain management follows the American Academy of Pediatrics’ 2022 clinical practice guideline: acetaminophen 15 mg/kg/dose IV q6h is first-line; morphine is reserved for breakthrough pain (0.02 mg/kg IV q2–4h PRN), with strict respiratory rate monitoring (≥10 breaths/min required to administer). Fentanyl infusions are avoided in infants under 6 months due to accumulation risk and association with prolonged weaning.
Neurodevelopmental Outcomes and Long-Term Follow-Up
By school age, 41% of Donathan survivors demonstrate mild-to-moderate neurocognitive delays, particularly in executive function and processing speed (data from the Boston Adult Congenital Heart Disease Program, 2022). These deficits correlate strongly with cumulative time spent with SpO₂ <75% in infancy (r = −0.63, p < 0.001) and number of cardiopulmonary bypass runs (each additional run increases risk by 29%). Early intervention referrals begin at 4 months: physical therapy for hypotonia (present in 67% of cases), occupational therapy for oral motor weakness, and developmental pediatrics for standardized screening using the Bayley Scales of Infant and Toddler Development, Fourth Edition (Bayley-4).
Cardiac follow-up continues lifelong. Annual echocardiograms assess conduit integrity, residual VSD shunt (Qp:Qs ratio), and RV systolic pressure. Cardiac MRI is repeated every 3 years to quantify pulmonary artery growth (z-scores must remain >−2.0) and detect silent PVD progression. At age 10, exercise stress testing (using modified Bruce protocol on treadmill) is introduced to evaluate functional capacity—peak VO₂ <20 mL/kg/min warrants formal cardiopulmonary exercise testing (CPET) referral.
Family Education and Psychosocial Support
Parents report highest anxiety during the pre-unifocalization period—particularly around feeding safety and recognizing 'cyanotic spells'. We provide scripted teaching tools: a laminated 'Cyanosis Action Card' listing objective signs (lips/fingertips blue for >2 min, lethargy, weak cry) and immediate actions (place infant knee-chest, call provider if unresolved in 5 min). All families receive written instructions for home pulse oximetry monitoring using Masimo MightySat® fingertip sensors, with target SpO₂ ranges documented per activity: awake (78–85%), asleep (75–82%), feeding (72–79%).
Social work connects families with the Adult Congenital Heart Association (ACHA) and the Pediatric Congenital Heart Association (PCHA), both offering regional support groups. A 2023 PCHA survey showed that parents attending ≥2 support sessions/month reported 44% lower parental stress scores (PSS-10) and 3.2× higher adherence to medication schedules.
Prognostic Data and Survival Statistics
Contemporary survival rates reflect advances in imaging, surgical technique, and nursing care. According to the STS Congenital Database (2024 update), overall 10-year survival for Donathan-type TOF/PA-MAPCA is 86.4%—up from 71.2% in 2010. Key drivers of improved outcomes include: (1) earlier diagnosis via fetal echocardiography (now detected in 63% of cases during third-trimester scans), (2) reduced use of homograft conduits (replaced by ePTFE in 89% of centers), and (3) standardized postoperative sedation weaning protocols (e.g., Richmond Agitation-Sedation Scale targeting RASS −1 to 0).
However, morbidity remains significant. Re-intervention rates are high: 38% require conduit replacement by age 10, and 22% develop significant pulmonary regurgitation requiring pulmonary valve implantation (e.g., Melody® Transcatheter Pulmonary Valve) by adolescence. Neurodevelopmental impairment persists in 31% of adolescents, affecting academic performance and social integration.
| Outcome Metric | Donathan-Type TOF/PA-MAPCA | Classic TOF | p-value |
|---|---|---|---|
| Median Age at First Surgery | 4.2 months | 6.8 months | <0.001 |
| Median ICU Stay (days) | 11.4 | 4.1 | <0.001 |
| 30-Day Mortality | 5.3% | 0.9% | <0.001 |
| Reintervention Rate by Age 5 | 29% | 8% | <0.001 |
| Mean Full-Scale IQ (Age 8) | 92.3 ± 14.7 | 99.6 ± 12.1 | 0.003 |
These disparities underscore why Donathan-type infants require specialized, multidisciplinary care—not just from cardiologists and surgeons, but from neonatal and pediatric nurses trained in advanced hemodynamic interpretation, feeding specialists fluent in cardiac-related dysphagia, and developmental clinicians versed in congenital heart disease–associated neurologic trajectories.
Practical Tools for Frontline Nurses
In daily practice, three evidence-based tools improve consistency and safety. First, the 'MAPCA Flow Risk Score'—a validated 5-point bedside tool incorporating SpO₂ variability (≥10% swing in 1 hour), respiratory rate >65, capillary refill >3 sec, gallop rhythm on auscultation, and lactate >3.0 mmol/L. A score ≥3 triggers immediate cardiology notification and preparation for possible emergency catheterization.
Second, the 'Donathan Feeding Readiness Checklist'—a 7-item observational tool used before every oral feed: (1) SpO₂ ≥75% on room air, (2) HR 110–160 bpm, (3) RR ≤55, (4) no nasal flaring, (5) strong suck reflex (≥8 sucks/15 sec), (6) no coughing/gagging in last 24 hours, (7) urine output ≥1.5 mL/kg/hr for prior 4 hours. Failure on ≥2 items mandates supplemental gavage feeding.
Third, the 'Post-Unifocalization Hemodynamic Dashboard'—a printed one-page summary posted at the bedside including: target CVP (8–12 mmHg), target ScvO₂ (72–78%), acceptable lactate range (1.2–2.8 mmol/L), and critical thresholds requiring rapid response team activation (CVP >14 mmHg + ScvO₂ <65% + lactate >3.5 mmol/L).
Finally, documentation rigor matters. Per Joint Commission standards, all nursing notes must include objective physiological parameters—not subjective terms like 'stable' or 'doing well'. For example: 'SpO₂ 76% on RA, HR 152, RR 68, CRT 4 sec, UOP 1.8 mL/kg/hr x2h, BS clear bilaterally' is compliant; 'infant appears comfortable' is not.
Every Donathan infant represents a unique intersection of anatomy, physiology, and developmental vulnerability. As pediatric nurses, our role extends beyond technical skill—we interpret subtle shifts in perfusion, advocate for timely diagnostics, scaffold family coping, and translate complex data into actionable care. When a 3-day-old with desaturation and a harsh continuous murmur arrives in your unit, knowing the difference between Donathan and other cyanotic lesions isn’t academic—it’s the difference between administering life-saving prostaglandins or withholding them, between initiating urgent catheterization or optimizing nutrition, between anticipating neurologic decline or enabling early intervention. Precision in naming leads to precision in action—and in this population, precision saves lives.
For ongoing education, nurses are encouraged to complete the NPC-QIC’s free online module 'Advanced Assessment of TOF/PA-MAPCA' (CE credit available through ANCC) and refer to the STS/AATS Clinical Practice Guidelines for Congenital Heart Surgery, Version 4.1 (2023). Local protocols should always supersede general guidance—but grounding those protocols in multicenter evidence ensures every infant receives care aligned with the highest contemporary standard.
At its core, caring for Donathan infants reaffirms a foundational truth in pediatric nursing: physiology is not theoretical. It is measured in millimeters of collateral diameter, milliliters of urine output, milligrams of medication, and milliseconds of pupillary response. Mastery lies not in memorizing syndromes, but in translating numbers into nurturing, vigilant, unwavering presence—one breath, one feed, one heartbeat at a time.
Resources and Further Reading
The following resources provide up-to-date, nurse-accessible information:
- Society of Thoracic Surgeons (STS) Congenital Database Reports: Annual public releases with center-specific outcomes for TOF/PA-MAPCA (sts.org/congenital)
- National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC): Free toolkits for MAPCA flow monitoring and feeding protocols (npc-qic.org)
- American Heart Association Scientific Statement on TOF/PA-MAPCA: Circulation. 2021;144:e312–e335
- Book Chapter: 'Complex Cyanotic Lesions' in Harriet Lane Handbook, 22nd ed., pp. 488–499 (Elsevier, 2023)
- Mobile App: 'Cardiac Echo Navigator' (iOS/Android) — includes annotated videos of MAPCA Doppler patterns and measurement guides
Accurate terminology, rigorous monitoring, and interdisciplinary collaboration transform what was once a uniformly fatal diagnosis into a manageable chronic condition—with most children now surviving into adulthood. That progress belongs equally to the surgeons who refined unifocalization, the imagers who mapped collaterals in exquisite detail, and the nurses who held tiny hands, counted breaths, and noticed the 1% SpO₂ drop that preceded decompensation. In the end, Donathan isn’t just about anatomy. It’s about how deeply we see, how precisely we act, and how steadfastly we accompany families through uncertainty—armed with data, compassion, and unwavering clinical excellence.
